Hemiplegia is a nonprogressive disorder that results in paralysis on one side of the body and is caused by brain or spinal cord trauma. Depending on the location and severity of the injury, the degree of hemiplegic symptoms varies. Congenital hemiplegia refers to the onset of hemiplegia before, during, or within the first two years of life. Acquired hemiplegia is a term used to describe hemiplegia occurring later in life [1].
Hemiplegia is caused by conditions such as stroke, brain infections (by bacteria, fungi, or viruses), brain trauma, brain tumors, and rare mutation in genes (alternating hemiplegia). Hemiplegia is a more general name for cerebral palsy (CP), which develops before birth and manifests in the first few years of life. Other hemiplegia types include alternating, facial, spinal, contralateral, spastic, and spastic hemiplegia [1,2].
Depending on the degree, hemiplegia symptoms may include muscle stiffness or weakness on one side, spasticity or permanently clenched muscles, poor fine motor skills, difficulty walking, unsteadiness, and difficulty grasping objects. Additionally, hemiplegic children are less active and require more developmental years than healthy children do. Additionally, they are permitted to play with just one hand or to hold one hand in a fist. If brain injury results in hemiplegia, brain damage may result in symptoms other than hemiplegia, such as memory loss, difficulty focusing, speech problems, behavioral disorders, and seizures. Various treatment modalities are available based on severity such as physiotherapy, multidisciplinary rehabilitation such as physical therapy, mental health therapist, and other medical management [1]. This review was conducted to oversee the different interventional therapies and their effects on hemiplegia pediatric patients.
National Library of Medicine: Pediatric Patients With Hemiplegia: A Systematic Review of a Randomized Controlled Trial – PMC (nih.gov)
In wheelchairs, individuals with CP hemiplegia sometimes punt with a foot or propel with one hand. Building on their efficiency is important. With foot punting, the floor to seat height is critical. Keeping the wheelchair frame width close to the users’ body helps with efficiency. Reduce seat dump. A power wheelchair with the controls set on the dominant side can help to preserve a mid-line posture. Some seat dump may be used for those who foot punt to reduce the forward migration of the pelvis into posterior pelvic tilt. Sometimes a trunk lateral support on the weaker side can help with stability.
Spastic diplegia/diparesis involves muscle stiffness that is mainly in the legs and less severely affects the arms and face, although the hands may be clumsy. Tendon reflexes in the legs are hyperactive. Tightness in certain leg muscles makes the legs move like the arms of a scissor. Children may require a walker or leg braces. Intelligence and language skills are usually normal.
National Institute of Neurological Disorders and Stroke: Cerebral Palsy | National Institute of Neurological Disorders and Stroke (nih.gov)
In wheelchairs, individuals with CP diplegia will often require a medial thigh support to help separate the knees. The user may also present with an anterior tilted pelvis requiring the backrest to be positioned more upright (closer to 90˚ hip angle).
Quadriplegia, also known as tetraplegia, is a severe form of paralysis that affects all four limbs, usually caused by a spinal cord injury. This debilitating condition has a profound impact on the lives of individuals who experience it, as well as their families and caregivers. In this article, we will delve into the causes, symptoms, and treatment options for quadriplegia, explore the challenges faced by quadriplegics, and shed light on the advancements in medical research and technology that offer hope for a better future. Quadriplegia is primarily caused by a traumatic injury to the cervical region of the spinal cord. This region, which includes the neck and upper back, is responsible for transmitting signals between the brain and the rest of the body. When the spinal cord is damaged, these signals are interrupted, resulting in the loss of sensation and motor control in all four limbs. The symptoms of quadriplegia can vary depending on the level and extent of the spinal cord injury. In addition to loss of movement and sensation, quadriplegics may experience respiratory difficulties, bladder and bowel dysfunction, changes in blood pressure, and increased susceptibility to secondary health complications like urinary tract infections and pressure sores. These physical challenges require careful management and specialized medical care [1].
Quadriplegia not only affects the physical functioning of individuals but also has a significant impact on their emotional and psychological well-being. Adjusting to life with quadriplegia can be a difficult and emotionally taxing process, as individuals may face feelings of grief, anger, frustration, and depression. The support of mental health professionals, family, and peers becomes crucial in navigating these emotional challenges. Rehabilitation plays a vital role in the recovery and long-term management of quadriplegia. Physical therapy, occupational therapy, and speech therapy are essential components of the rehabilitation process, helping quadriplegics regain functional independence, improve muscle strength, and enhance their communication abilities. Assistive devices such as wheelchairs, mobility aids, voice-activated technology, and environmental control systems are instrumental in facilitating greater mobility and independence. Living with quadriplegia presents numerous challenges that extend beyond the physical limitations. Accessibility issues in public spaces, transportation limitations, financial constraints, and societal stigmas often hinder the full participation and inclusion of quadriplegics in various aspects of life. These challenges underscore the need for greater awareness, policy changes, and infrastructure improvements to ensure equal opportunities for individuals with quadriplegia [2].
Advancements in medical research and technology have paved the way for innovative solutions to address the challenges faced by quadriplegics. Neuroprosthetics, brain-computer interfaces, exoskeletons, and robotic assistive devices offer hope for restoring limb functionality and improving mobility. Furthermore, emerging techniques such as spinal cord stimulation and regenerative medicine hold promise for repairing damaged neural tissue and restoring motor function. Psychological support and coping strategies are integral to enhancing the quality of life for quadriplegics. Counseling, peer support groups, and adaptive sports and recreation programs can provide individuals with quadriplegia with emotional support, a sense of belonging, and opportunities for personal growth and achievement. Additionally, cultivating resilience, practicing mindfulness, and engaging in creative outlets are valuable coping mechanisms for navigating the challenges associated with quadriplegia. The voices of quadriplegics and their advocates are crucial in raising awareness, influencing policy changes, and promoting inclusivity. Advocacy efforts focus on improving accessibility, healthcare services, employment opportunities, and societal attitudes towards disability. By empowering quadriplegics to actively participate in decision-making processes and encouraging their self-advocacy, we can create a more inclusive and supportive society [3].
Hilaris Publishing SRL: Quadriplegia: Understanding the Challenges and Pursuing Hope (hilarispublisher.com)
While individuals with CP quadriplegia vary in severity & presentation, normally they would need a lot of support & asymmetrical contouring to adapt to their body shape. Some components may be used to correct while others are used to accommodate the user’s tone. Check for dominant side for vision & hearing as this may dictate the sitting position. Also, some users when in power chairs, can change their sitting position to drive. it is critical to test the user statically & when driving for a complete overview.
Cerebral palsy is the most frequent cause of severe physical disability in childhood. Dyskinetic cerebral palsy (DCP) is the second most common type of cerebral palsy after spastic forms. DCP is typically caused by non-progressive lesions to the basal ganglia or thalamus, or both, and is characterised by abnormal postures or movements associated with impaired tone regulation or movement coordination. In DCP, two major movement disorders, dystonia and choreoathetosis, are present together most of the time. Dystonia is often more pronounced and severe than choreoathetosis, with a major effect on daily activity, quality of life, and societal participation. The pathophysiology of both movement disorders is largely unknown. Some emerging hypotheses are an imbalance between indirect and direct basal ganglia pathways, disturbed sensory processing, and impaired plasticity in the basal ganglia. Rehabilitation strategies are typically multidisciplinary.
The Lancet: Clinical presentation and management of dyskinetic cerebral palsy – The Lancet Neurology
Individuals with Athetoid CP may prefer added support & have their arms & feet strapped to control unwanted involuntary movements. When using a head control diving facility, it is critical to stabilize the body & limbs in view of more fine motor control.
Like all other types of cerebral palsy, ataxic CP is a developmental disorder that affects motor function. Ataxic cerebral palsy is characterized by problems with balance and coordination. These issues are caused by damage to the developing brain’s motor control centers.
Children with ataxic cerebral palsy have a hard time controlling their movements. They are shaky and struggle with precise movements, such as writing and grasping small objects. Ataxic CP can affect the hands, arms, legs, feet, eyes and even speech.
Cerebral Palsy Guidance: Ataxic Cerebral Palsy – Causes, Symptoms and Treatment (cerebralpalsyguide.com)
Severe cases of ataxic CP are often not able to walk independently & require a wheelchair or mobility aid. Keeping the wheelchair lightweight & well-fitting can help maximize the ability of the user. The seating may require some adaptability to conform to the user or help correct posture.
Hypotonic cerebral palsy is a developmental disorder that affects motor function. Like all other types of cerebral palsy, this form of cerebral palsy is caused by brain damage before, during, or shortly after childbirth. Hypotonic cerebral palsy is one of the rarest types of the condition, accounting for 2.6% of all cases.
Hypotonic cerebral palsy is generally characterized by low muscle tone, resulting in floppy muscles. The instability of muscles can cause children to miss important developmental milestones such as rolling over, sitting up, crawling, and walking. The floppy muscles do not lack strength but instead lack stability.
Cerebral Palsy Guide: Hypotonic Cerebral Palsy – Causes, Symptoms and Treatment (cerebralpalsyguide.com)
Individuals with low underlying muscle tone (Hypotonia) often have difficulty maintaining an upright posture & head control.
Cerebrovascular accident (CVA), otherwise called a stroke, is the third major cause of morbidity and mortality in many developed countries. Stroke can be either ischemic or hemorrhagic. Ischemic stroke is due to the loss of blood supply to an area of the brain. It is a common type of stroke.
Hemorrhagic stroke is due to bleeding into the brain by the rupture of a blood vessel. Hemorrhagic stroke may be further subdivided into intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH). ICH is bleeding into the brain parenchyma, and SAH is bleeding into the subarachnoid space. Hemorrhagic stroke is associated with severe morbidity and high mortality.[1] Progression of hemorrhagic stroke is associated with worse outcomes. Early diagnosis and treatment are essential given the usual rapid expansion of hemorrhage, causing sudden deterioration of consciousness and neurological dysfunction.
National Library of Medicine / National Institutes of Health: Hemorrhagic Stroke – StatPearls – NCBI Bookshelf (nih.gov)
Depending on the level of severity a stroke patient may have partial paralysis, often down one side presenting similar to hemiplegia (see hemiplegia). Less mobile patients may use a powerchair & require pressure management & a wheelchair with tilt to relieve pressure. Giving the head some lateral support may help with head control. Maintain postural alignment & an upright posture with supports.
A transient ischemic attack (TIA) is a medical emergency. It is defined as a transient episode of neurologic dysfunction due to the focal brain, spinal cord, or retinal ischemia without acute infarction or tissue injury. The definition of a TIA has moved from time-based to tissue based. A TIA typically lasts less than an hour, more often minutes. TIA can be considered a serious warning of an impending ischemic stroke; the risk is highest in the first 48 hours following a transient ischemic attack. Differentiating transient ischemic attack from other mimicking conditions is important. Transient ischemic attacks are usually associated with a focal neurologic deficit and/or speech disturbance in a vascular territory due to underlying cerebrovascular disease. It is always sudden in onset. Evaluation of TIA should be done urgently with imaging and laboratory studies to decrease the risk of subsequent strokes. The subsequent risk of TIA or ischemic stroke can be stratified with a simple clinical measure. Immediate multimodality therapeutic interventions should be initiated. These will include aggressive treatment of blood pressure, high-dose statin, antiplatelet therapy, blood sugar control, diet, and exercises. Specific underlying etiology needs to be managed accordingly. This treatment scheme may substantially reduce the risk of recurrent strokes or future TIA by at least 80%.[1][2][3]
National Library of Medicine / National Institutes of Health: Transient Ischemic Attack – StatPearls – NCBI Bookshelf (nih.gov)
Multiple sclerosis (MS) is a chronic autoimmune, inflammatory neurological disease of the central nervous system (CNS).1,2 MS attacks the myelinated axons in the CNS, destroying the myelin and the axons to varying degrees.3,4
The course of MS is highly varied and unpredictable. In most patients, the disease is characterized initially by episodes of reversible neurological deficits, which is often followed by progressive neurological deterioration over time.
The cause is unknown, but it appears to involve a combination of genetic susceptibility and a nongenetic trigger, such as a virus, metabolism, or environmental factors, that together result in a self-sustaining autoimmune disorder that leads to recurrent immune attacks on the CNS.7
Neurologists agree that patients may be grouped into four major categories based on the course of disease:2
Relapsing–remitting MS: the most common form, affecting about 85% of MS patients. It is marked by flare-ups (relapses or exacerbations) of symptoms followed by periods of remission, when symptoms improve or disappear.
Secondary progressive MS: may develop in some patients with relapsing–remitting disease. For many patients, treatment with disease-modifying agents helps delay such progression. The disease course continues to worsen with or without periods of remission or leveling off of symptom severity (plateaus).
Primary progressive MS: affects approximately 10% of MS patients. Symptoms continue to worsen gradually from the beginning. There are no relapses or remissions, but there may be occasional plateaus. This form of MS is more resistant to the drugs typically used to treat the disease.
Progressive-relapsing MS: a rare form, affecting fewer than 5% of patients. It is progressive from the start, with intermittent flare-ups of worsening symptoms along the way. There are no periods of remission.
National Library of Medicine / National Institutes of Health: Multiple Sclerosis Review – PMC (nih.gov)
Comfortable seating with good pressure management should be considered with Multiple Sclerosis. Lateral Trunk contouring can be helpful to keep the user in the midline position. Neutral seat dump & knee separation with good medial thigh contouring can aid with maintaining the pelvis & a better spinal posture.
Duchenne muscular dystrophy (DMD) usually presents in early childhood with delayed motor milestones including delays in walking independently and standing up from a supine position. Proximal weakness causes a waddling gait and difficulty climbing stairs, running, jumping, and standing up from a squatting position. DMD is rapidly progressive, with affected children being wheelchair dependent by age 12 years. Cardiomyopathy occurs in almost all individuals with DMD after age 18 years. Few survive beyond the third decade, with respiratory complications and progressive cardiomyopathy being common causes of death.
National Library of Medicine / National Institutes of Health: Dystrophinopathies – GeneReviews® – NCBI Bookshelf (nih.gov)
The future needs to be considered With Muscular Dystrophy as the user will progressively become weaker requiring more external support. Initially the user may have a self-propelling chair then as the condition deteriorates, move towards a powerchair. In the ambulatory phase the user may develop an anterior pelvic tilt to compensate the developing weakness which may need to be considered in the wheelchair seat in terms of back support & Pelvic positioning. Comfort & pressure management are essential as well as support. Gel is a good consideration as it attempts to replace the consistency & support of atrophied muscle tissue creating a firm surface compared to foam.
Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving a mutation in the dystrophin gene. Progressive muscle weakness, most notably of the proximal lower limbs, is the primary manifestation of this condition.[1][2] The onset of BMD symptoms varies widely between 5 and 60 years. In a study involving 67 individuals with BMD, most were found ambulant until their 40s or older, while a small group experienced earlier loss of ambulation.[3] BMD symptoms usually appear at a later age than a similar X-linked disorder, Duchenne muscular dystrophy (DMD).
DMD and BMD may be easier to distinguish based on the age at which patients become wheelchair-dependent. Patients with DMD are wheelchair-dependent before age 13, while individuals with BMD may remain ambulatory even after age 16. Patients presenting with proximal muscle weakness before turning 12 may be hard to diagnose without genetic analysis.[4]
BMD is currently considered a mild form of DMD rather than a distinct clinical entity. Consequently, interventional trials are more rarely conducted for BMD than DMD.[5]
National Library of Medicine / National Institutes of Health: Becker Muscular Dystrophy – StatPearls – NCBI Bookshelf (nih.gov)
Facioscapulohumeral muscular dystrophy (FSHD) typically presents with weakness of the facial muscles, the stabilizers of the scapula, or the dorsiflexors of the foot. Severity is highly variable. Weakness is slowly progressive and approximately 20% of affected individuals eventually require a wheelchair. Life expectancy is not shortened.
National Library of Medicine / National Institutes of Health: Facioscapulohumeral Muscular Dystrophy – GeneReviews® – NCBI Bookshelf (nih.gov)
Myotonic dystrophy (DM) is considered a subgroup of myopathy and the most common type of muscular dystrophy that begins in adulthood. There are two major forms recognized based on clinical and molecular presentation: Myotonic dystrophy type I (DM1), known as Steinert disease, and myotonic dystrophy type II (DM2), or proximal myotonic myopathy which is a milder variety of DMI. These are progressive, multisystem genetic disorders. Clinical presentation is diverse and can range from asymptomatic electrical myotonia to severe weakness and disability, including cardiac conduction defects, infertility, cataracts, and insulin resistance.[1] A congenital form of DM type 1 is associated with an apparent developmental disability. Myotonia is characterized by impaired relaxation of muscles after voluntary contraction due to repetitive depolarization of the muscle membrane. Myotonia, due to myotonic dystrophy, improves with repeated exercise and is worsened by exposure to cold.
National Library of Medicine / National Institutes of Health: Myotonic Dystrophy – StatPearls – NCBI Bookshelf (nih.gov)
Monoplegia is a type of paralysis that impacts one limb, such as an arm or leg on one side of your body. This happens when damage to a part of the nervous system disrupts nerve signaling to the muscles in the affected limb.
Monoplegia can affect the upper or lower body, either one arm or one leg. Symptoms can appear suddenly or progress gradually over time.
Monoplegia is often caused by cerebral palsy. But it can also be the result of an injury or trauma to the brain, spinal cord, or the affected limb.
Although monoplegia can sometimes improve over time, it may be permanent in some individuals. Treatment options typically focus on alleviating symptoms and improving quality of life.
Healthline: Monoplegia: What It Is, Causes, Symptoms & Treatment (healthline.com)
See Hemiplegia
Hemiplegia is the medical term for paralysis of one side of the body. It results in muscular wasting on the affected side, impairs gait, reduces motor abilities, and causes instability and a loss of grasping capacity. The patient’s quality of life is impacted by hemiplegia because it impairs brain and spinal cord functions. Consequently, a range of therapeutic options, including physical therapy, medical health management, and other multidisciplinary care, are accessible.
National Library of Medicine / National Institutes of Health: Pediatric Patients With Hemiplegia: A Systematic Review of a Randomized Controlled Trial – PMC (nih.gov)
In wheelchairs, these users sometimes punt with a foot or propel with one hand. Building on their efficiency is important. With foot punting, the floor to seat height is critical. Keeping the wheelchair frame width close to the user’s body helps with efficiency. Reduce seat dump. A power wheelchair with the controls set on the dominant side can help to preserve a mid-line posture. Some seat dump may be used for those who foot punt to reduce the forward migration of the pelvis into posterior pelvic tilt. Sometimes a trunk lateral support on the weaker side can help with stability.
Paraplegia is the symptom of paralysis that mainly affects your legs (though it can sometimes affect your lower body and some of your arm abilities, too). This usually happens because of injuries to your nervous system, especially your spinal cord, but it can also happen with various medical conditions and diseases.
Cleveland Clinic: Paraplegia: Definition, Causes, Symptoms, Types & Treatment (clevelandclinic.org)
Due to reduced muscle tone, atrophied muscle & reduced sensation with paralysis, it is essential to provide a high degree of pressure management. Seating which enables easy transfers & which provides stability to the pelvis & trunk to maximize efficiency.
Quadriplegia is a clinical sign consisting of the complete loss of motor power in all four extremities. It is most often due to pathology of the central or peripheral nervous system or muscle disease; very seldom it is psychogenic. High spinal cord lesions, multiple peripheral nerve involvement as in the Guillain–Barré syndrome, or widespread muscle dysfunction as in hypokalemic periodic paralysis can lead to quadriplegia. Treatment depends on the etiology.
Science Direct: Quadriplegia – an overview | ScienceDirect Topics
Due to reduced muscle tone, atrophied muscle & reduced sensation with paralysis, it is essential to provide a high degree of pressure management. Quadriplegics often use powerchairs & require more lateral support & stability.
A simple meningocele comprises of meninges and CSF protruded into the subcutaneous tissue through a spinal defect. Skin overlying meningocele is usually intact. A complex meningocele is associated with other spinal anomalies. Meningocele is a typically asymptomatic spinal anomaly and is not associated with acute neurologic conditions. Neural tube defects are the second most common type of congenital disability after congenital heart defects.
National Library of Medicine / National Institutes of Health: Meningocele – StatPearls – NCBI Bookshelf (nih.gov)
Spinabifida users are often active & desire independence. A special setup of the cushion & backrest to accommodate/correct an asymmetrical body shape or posture may be required. With paralysis, a pressure management cushion should be considered as well as ability to transfer.
Neural tube defects are a spectrum of congenital anomalies that include cranial defects and open or closed spinal dysraphism. Open spinal dysraphisms or spina bifida aperta are often seen during the physical examination as visible lesions and include malformations such as myelomeningocele. On the other hand, closed spinal dysraphisms or spina bifida occulta are non-visible lesions, usually with no apparent signs recognized during physical examination. Thus, patients are often asymptomatic with no future complications and usually, do not require treatments. Myelomeningocele generally occurs during embryonic development due to incomplete closure of the spinal neural tube during the first month of pregnancy. It ultimately leads to an exposed neural tissue or meninges with a fluid-filled sac that protrudes at the affected vertebral level.[1][2][3] Myelomeningocele can cause several neurological deficits and complications which are dependent on the involved vertebral site. It can lead to devastating morbidity and multiple disabilities; hence, the prognosis is often worse if diagnosed late or left untreated.[4][5]
National Library of Medicine / National Institutes of Health: Myelomeningocele – StatPearls – NCBI Bookshelf (nih.gov)
Angelman syndrome (AS) is characterized by severe developmental delay or intellectual disability, severe speech impairment, gait ataxia and/or tremulousness of the limbs, and unique behavior with an apparent happy demeanor that includes frequent laughing, smiling, and excitability. Microcephaly and seizures are also common. Developmental delays are first noted at around age six months; however, the unique clinical features of AS do not become manifest until after age one year.
National Library of Medicine / National Institutes of Health: Angelman Syndrome – GeneReviews® – NCBI Bookshelf (nih.gov)
Angelman Syndrome users may present with scoliosis & have hyperactive lower extremity deep tendon reflexes. Support may be required to prevent deterioration of a scoliosis & a slightly open hip angle may help with constipation.
Friedreich ataxia (FA) is the most common hereditary ataxia accounting for approximately 50% of all ataxia cases.[1][2][3][4][5] It was first reported in 1863 by the German physician Nikolaus Friedreich. The disease causes neurodegeneration and manifests as a combination of difficulty in ambulation, muscle weakness, loss of sensation and proprioception, and impaired speech.[6][7][8][9][10] It has an autosomal recessive inheritance pattern, and symptom onset is usually in childhood. Unfortunately, symptoms worsen as time progresses, so most people affected by this disease end up requiring mobility aids such as wheelchairs, lose their vision and hearing, and develop other medical complications such as diabetes mellitus and scoliosis
National Library of Medicine / National Institutes of Health: Friedreich Ataxia – StatPearls – NCBI Bookshelf (nih.gov)
Pressure management for muscle atrophy, maximizing efficiency & external support to prevent scoliosis should be considered with Friedreich’s Ataxia as well as consideration for self-transfers.
Osteogenesis imperfecta (OI) is a genetic disorder of connective tissues caused by an abnormality in the synthesis or processing of type I collagen.[1][2] It is also called brittle bone disease. It is characterized by an increased susceptibility to bone fractures and decreased bone density. Other manifestations include blue sclerae, dentinogenesis imperfecta, short stature, as well as deafness in adulthood. There are also reports of valvular insufficiencies and aortic root dilation. Milder manifestations include generalized laxity, easy bruising, hernias, and excess sweating.[3] Clinical manifestations range from mild with a nearly asymptomatic form to most severe forms (involving infants presenting with crumpled ribs, fragile cranium, and long bone fractures incompatible with life), resulting in increased perinatal mortality.[4]
National Library of Medicine / National Institutes of Health: Osteogenesis Imperfecta – StatPearls – NCBI Bookshelf (nih.gov)
Osteogenesis imperfecta wheelchair users may either self-propel or use a powerchair. Care must be taken with seating supports to prevent fractures. Often the user may self-transfer to & from the wheelchair so the ability to lower the wheelchair seat may help. External lateral supports can help to reduce the risk of scoliosis.
Winchester syndrome is an extremely rare congenital connective tissue and bone disorder. Winchester syndrome is characterized most frequently by short stature, wearing down of bone and tissue, dark skin patches and coarse facial features. The main feature of this syndrome is short stature due to changes in the vertebrae of the backbone and long bones of the limbs that get worse over time (degenerative). Other symptoms commonly include arthritis-like symptoms, loss of bone tissue (osteolysis), reduced bone density (osteoporosis), nodules under the skin (subcutaneous), coarse facial features and abnormalities of the eyes and teeth. The most commonly affected joints are those of the hands, feet, knees, shoulders, elbow and hip. Winchester syndrome is caused by changes (variants or mutations) in the MMP14 gene and it is inherited in a recessive manner.
National Organization for Rare Disorders: Winchester Syndrome – Symptoms, Causes, Treatment | NORD (rarediseases.org)
Winchester syndrome often includes progressive painful arthropathy affecting many joints. Bones become brittle & prone to fracture. Consider comfortable seating which can conform to the asymmetrical shape of the user.
McCune-Albright syndrome is a rare genetic disordered originally recognized by the triad of polyostotic fibrous dysplasia, precocious puberty, and café-au-lait spots. A variety of endocrine disorders, including hyperthyroidism, acromegaly, phosphate wasting, and Cushing syndrome are now considered as part of the endocrinopathies seen in this disorder. The variable constellation of symptoms arises from a somatic activating mutation of the GNAS gene, which is present in many tissue types.[1]
National Library of Medicine / National Institutes of Health: McCune-Albright Syndrome – StatPearls – NCBI Bookshelf (nih.gov)
A wheelchair can make mobility easier for some with McCune-Albright syndrome (MAS). Some may experience a high degree of deformity requiring asymmetrically adaptable seating. Bones can break easily so care must be taken when transferring in & out of the wheelchair. Supporting & maintaining posture with softer surfaces may help to minimize bone pain.
Clinical description: A-T is a complex disorder with substantial variability in the severity of features between affected individuals, and at different ages. Neurological symptoms most often first appear in early childhood when children begin to sit or walk. They have immunological abnormalities including immunoglobulin and antibody deficiencies and lymphopenia. People with A-T have an increased predisposition for cancers, particularly of lymphoid origin. Pulmonary disease and problems with feeding, swallowing and nutrition are common, and there also may be dermatological and endocrine manifestations.
National Library of Medicine / National Institutes of Health: Ataxia telangiectasia: a review – PubMed (nih.gov)
People with Ataxia-telangiectasia may initially use a self-propelling wheelchair but as it progresses a powerchair may be required. Support is needed to maintain a mid-line posture & maximize fine motor function. As the atrophy progresses in the muscles, cushions with pressure management need to be considered.
Leukodystrophies are a group of rare neurological (nervous system) diseases. They affect the white matter in the brain and spinal cord. White matter is tissue made of insulated nerve fibers.
Leukodystrophies target myelin, which is the protective insulation covering nerve cells. Without myelin, nerves can’t communicate well. Leukodystrophies lead to a progressive loss of neurological function. The brain and the body can’t receive signals from each other. These diseases are often fatal.
Cleveland Clinic: Leukodystrophy: What is it, Types, Symptoms, Treatment & Outlook (clevelandclinic.org)
Increasing postural support in the wheelchair may be needed to maximize fine motor function & overtime the pressure management needs to be considered for reduced muscle tone.
Polio is a highly infectious disease caused by a virus. It invades the nervous system and can cause total paralysis in a matter of hours. The virus is transmitted by person-to-person spread mainly through the faecal-oral route or, less frequently, by a common vehicle (for example, contaminated water or food) and multiplies in the intestine. Initial symptoms are fever, fatigue, headache, vomiting, stiffness of the neck and pain in the limbs. One in 200 infections leads to irreversible paralysis (usually in the legs). Among those paralysed, 5–10% die when their breathing muscles become immobilized.
Polio mainly affects children under 5 years of age. However, anyone of any age who is unvaccinated can contract the disease.
World Health Organization: Poliomyelitis (who.int)
With polio, like paralysis, maintaining a mid-line posture can reduce the chances of developing asymmetries. If a scoliosis has developed a backrest in which the contours can adapted to the asymmetrical body shape of the user may be used. With muscle atrophy, a cushion which can reduce localized pressure can be appropriate.
Arthritis is defined as an acute or chronic joint inflammation in the joint. Arthritis may attribute to a wide variety of symptoms that include pain, stiffness, decreased range of motion, and joint deformities. There are several different types of arthritis, with management being different for each. History and physical examination are crucial in recognizing the type of arthritis, while additional laboratory and imaging may sometimes be necessary for confirming the diagnosis. This activity outlines the evaluation and treatment of arthritis and explains the role of the interprofessional team in managing patients with this condition.
National Library of Medicine / National Institutes of Health: Arthritis – StatPearls – NCBI Bookshelf (nih.gov)
With Spinal Arthritis, maintaining an upright sitting posture with good spinal extension can help to reduce the pain & is the easiest posture to maintain for longer periods. When collapse or a slumped posture is allowed pain can be worse & transferring to & from the wheelchair may be difficult.
Ankylosing spondylitis (AS) is a chronic, inflammatory disease of the axial spine. Chronic back pain and progressive spinal stiffness are the most common features of this disease. Involvement of the spine, sacroiliac joints, peripheral joints, digits, and entheses are characteristic. Impaired spinal mobility, postural abnormalities, buttock pain, hip pain, peripheral arthritis, enthesitis, and dactylitis are all commonly associated with ankylosing spondylitis.
National Library of Medicine / National Institutes of Health: Ankylosing Spondylitis – StatPearls – NCBI Bookshelf (nih.gov)
“Cerebrovascular disease is an umbrella term for conditions that impact the blood vessels in your brain. “Cerebro” refers to your brain and “vascular” refers to your blood vessels (arteries and veins).
Cerebrovascular diseases may cause a reduction of blood flow to your brain (ischemia) or bleeding (hemorrhage) in a part of your brain. Both conditions are generally referred to as “stroke.” Blood vessel diseases in the brain can lead to strokes, as well as many other vascular conditions.”
Cleveland Clinic: Cerebrovascular Disease: Types, Causes & Symptoms (clevelandclinic.org)
See Stroke
Parkinson’s disease is a progressive disorder that affects the nervous system and the parts of the body controlled by the nerves. Symptoms start slowly. The first symptom may be a barely noticeable tremor in just one hand. Tremors are common, but the disorder also may cause stiffness or slowing of movement.
In the early stages of Parkinson’s disease, your face may show little or no expression. Your arms may not swing when you walk. Your speech may become soft or slurred. Parkinson’s disease symptoms worsen as your condition progresses over time.
Although Parkinson’s disease can’t be cured, medicines might significantly improve your symptoms. Occasionally, a health care professional may suggest surgery to regulate certain regions of your brain and improve your symptoms.
Mayo Clinic: Parkinson’s disease – Symptoms and causes – Mayo Clinic
With Parkinsons, it is good to have a low seat to floor height which enables self-propulsion and lowers the risk of falls. Sometimes individuals may need a powerchair if symptoms limit ability to propel.
Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), known as ALS, is a nervous system disease that affects nerve cells in the brain and spinal cord. ALS causes loss of muscle control. The disease gets worse over time.
ALS is often called Lou Gehrig’s disease after the baseball player who was diagnosed with it. The exact cause of the disease is still not known. A small number of cases are inherited.
ALS often begins with muscle twitching and weakness in an arm or leg, trouble swallowing or slurred speech. Eventually ALS affects control of the muscles needed to move, speak, eat and breathe. There is no cure for this fatal disease.
Mayo Clinic: Amyotrophic lateral sclerosis (ALS) – Symptoms and causes – Mayo Clinic
Many people with ALS spend most of their time in their wheelchairs, so the chairs must be comfortable and facilitate good health & promote good posture.
Motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy motor neurons, the cells that control skeletal muscle activity such as walking, breathing, speaking, and swallowing. This group includes diseases such as amyotrophic lateral sclerosis, progressive bulbar palsy, primary lateral sclerosis, progressive muscular atrophy, spinal muscular atrophy, Kennedy’s disease, and post-polio syndrome.
National Institute of Neurological Disorders and Stroke: Motor Neuron Diseases | National Institute of Neurological Disorders and Stroke (nih.gov)
There are two types of manual wheelchair that are used to meet the needs of people with MND when they are beginning to have mobility problems. One is a folding, portable wheelchair, with self-propelling or attendant wheels, and the other offers more support with a tilt-in-space mechanism. for others a powerchair may be required with attendant controls. A wheelchair for someone with MND should meet their current and future needs as the condition progresses. This will usually mean selecting a high specification wheelchair with a range of functions and postural supports. Initially some of these functions and supports may not be fully used, but over the course of disease progression they often become essential for the continued use of the wheelchair. Pressure management cushions and increased support together with tilt, recline & elevating leg functions are often used as the postural & functional needs change.
Diabetes is a chronic disease that occurs either when the pancreas does not produce enough insulin or when the body cannot effectively use the insulin it produces. Insulin is a hormone that regulates blood glucose. Hyperglycaemia, also called raised blood glucose or raised blood sugar, is a common effect of uncontrolled diabetes and over time leads to serious damage to many of the body’s systems, especially the nerves and blood vessels.
World Health Organization: Diabetes (who.int)
As diabetes progresses, many people find mobility aids to be useful. Diabetes affects millions of people worldwide and can cause fatigue and other issues that can be solved with the proper wheelchair. The most frequently mentioned wheelchair adjustments are related to neuropathies, skin integrity, decreased strength and amputations.
Progressive supranuclear palsy is a rare brain disease that affects walking, balance, eye movements and swallowing. The disease results from the damage of cells in areas of the brain that control body movement, coordination, thinking and other important functions. Progressive supranuclear palsy also is called Steele-Richardson-Olszewski syndrome.
Progressive supranuclear palsy worsens over time and can lead to dangerous complications, such as pneumonia and trouble swallowing. There’s no cure for progressive supranuclear palsy, so treatment focuses on managing the symptoms.
Mayo Clinic: Progressive supranuclear palsy – Symptoms and causes – Mayo Clinic
Comfortable pressure relieving cushion is often prescribed to reduce the risk of pressure injury developing. Contoured Support is also important to maintain good postural alignment countering the postural instability. The seating may need to be adjustable to adapt to the asymmetrical postural needs of the individual.
Dejerine-Sottas syndrome (DSS) is an inherited neurological condition that gradually affects the ability to move. Peripheral nerves are the nerves outside of the brain and spinal cord. These nerves become enlarged or thickened leading to muscle weakness. The condition may progress irregularly and can often be accompanied by pain, weakness, numbness, and a tingling, prickling or burning sensation in the legs. DSS can result in the inability to walk but is not thought to shorten lifespan. This condition does not impact brain function.
National Organization for Rare Disorders: Dejerine-Sottas Syndrome – Symptoms, Causes, Treatment | NORD (rarediseases.org)
With, Dejerine-Sottas disease, like other progressive conditions, pressure management cushions & increased postural support are important & may need increasing as the disease progresses.
Morquio syndrome is a rare inherited birth defect that is estimated to occur in one of every 200,000 births. The disease may not be visible at birth; symptoms usually begin between ages 1 and 3. Morquio syndrome is a progressive disease, meaning symptoms get worse as a child grows.
Morquio syndrome is part of a group of diseases called mucopolysaccharidosis (MPS). Morquio is also known as MPS IV.
In children with Morquio syndrome, the body cannot break down sugar chains called glycosaminoglycans that help build bone, cartilage, eye corneas, skin and connective tissue (such as tendons, ligaments, etc.)
People with glycosaminoglycans diseases either do not produce enough of the enzymes needed to break sugar chains into simpler molecules, or they produce enzymes that do not work normally. As a result, glycosaminoglycans collect in cells, blood and connective tissue and cause damage over time.
Children’s Hospital of Philadelphia: Morquio syndrome | Children’s Hospital of Philadelphia (chop.edu)
Children with spine and bone conditions can have difficulty walking and may require a wheelchair. Seating which can conform to the individual’s body shape are important due to the high risk of scoliosis & abnormal bone & spine development.
Pelizaeus-Merzbacher disease (PMD) is a rare X-linked genetic disorder affecting the central nervous system that is associated with abnormalities of the white matter of the brain and spinal cord. It is one of the leukodystrophies in which disease is due to abnormal development of one or more components (predominantly fats or proteins) that make up the white matter (myelin sheath) of the brain. The myelin sheath is the protective covering of the nerve and nerves cannot function normally without it. In PMD, many areas of the central nervous system may be affected, including the deep portions of the cerebrum (subcortical), cerebellum, brain stem and spinal cord. Signs may include the impaired ability to coordinate movement (ataxia), involuntary muscle spasms (spasticity) that result in slow, stiff movements of the legs, delays in reaching developmental milestones, late onset loss of motor abilities, and progressive deterioration of intellectual function. The neurologic signs of PMD are usually slowly progressive.
National Organization for Rare Disorders: Pelizaeus-Merzbacher Disease – Symptoms, Causes, Treatment | NORD (rarediseases.org)
Pelizaeus–Merzbacher disease may include the need for wheelchair seating, physical therapy, and orthotics to prevent or ameliorate the effects of scoliosis; special education; and assistive communication devices. Adaptable seating which can be adjusted to suit the changing needs of the individual.
Sandhoff disease is a rare lysosomal storage disease. It causes the destruction of nerve cells (neurodegeneration). This leads to problems with thinking and moving. Sandhoff disease is caused by harmful changes in the HEXB gene. Harmful changes in this gene cause decreased amounts of two enzymes in the recycling centers (lysosomes) of the cell. Without these enzymes, certain fats (lipids) build up in large amounts in the nerve cells. This damages the brain and spinal cord (central nervous system). Sandhoff disease is very similar to Tay Sachs disease.
National Organization for Rare Disorders: Sandhoff Disease – Symptoms, Causes, Treatment | NORD (rarediseases.org)
Like all progressive diseases, with Sandhoff’s disease, the wheelchair must meet current and future needs as the disease progresses.
Krabbe disease belongs to a group of disorders called leukodystrophies, rare genetic disorders that affect the white matter of the brain. Krabbe disease is characterized by a deficiency in the enzyme galactocerebrosidase (GALC) which is an enzyme that uses water molecules to break down certain fats (lipids). Failure to break down these lipids results in deterioration of the protective covering (myelin sheath) surrounding nerves in the brain (demyelination). Characteristic globoid (sphere shaped) cells appear in affected areas of the brain. These enlarged fatty cells accumulate in affected areas and cause a variety of progressive neurological symptoms. Depending on the age that symptoms begin, individuals may present with intellectual and behavioral delays, blindness, deafness, paralysis of facial muscles (pseudobulbar palsy), muscle spasms and seizures. Most patients are diagnosed by measuring GALC enzyme activity in leukocytes taken from a blood sample. Very low activity could indicate a diagnosis of Krabbe disease. In some states, diagnosis may occur through newborn screening. If newborns are diagnosed before 14 days old, they are candidates for early treatment with hematopoietic stem cell transplantation (HSCT). Krabbe disease is inherited in an autosomal recessive pattern.
National Organization for Rare Disorders: Krabbe Disease – Symptoms, Causes, Treatment | NORD (rarediseases.org)
With Krabbe’s disease, where there is severe spasticity & muscle weakness a wheelchair or stroller may be needed. The seat must be adjustable to the current & future needs of the individual.
Epilepsy — also known as a seizure disorder — is a brain condition that causes recurring seizures. There are many types of epilepsy. In some people, the cause can be identified. In others, the cause is not known.
Seizure symptoms can vary widely. Some people may lose awareness during a seizure while others don’t. Some people stare blankly for a few seconds during a seizure. Others may repeatedly twitch their arms or legs, movements known as convulsions.
Mayo Clinic: Epilepsy – Symptoms and causes – Mayo Clinic
Where there are seizures, ensure the head is supported, & if in a tilting wheelchair, use some tilt to prevent falls as well as a seat belt. Sometimes a dynamic backrest is useful as it can reduce the strain on the individual & wheelchair then return to its position afterwards.
A seizure is a sudden, uncontrolled burst of electrical activity in the brain. It can cause changes in behavior, movements, feelings and levels of consciousness. Having two or more seizures at least 24 hours apart that don’t have a known cause is considered to be epilepsy.
There are many types of seizures, and they have a range of symptoms and severity. Seizure types vary by where they begin in the brain and how far they spread. Most seizures last from 30 seconds to two minutes. A seizure that lasts longer than five minutes is a medical emergency.
Seizures can happen after a stroke or a head injury. They also may be caused by an infection such as meningitis or another illness. Many times, though, the cause is unknown.
Mayo Clinic: Seizures – Symptoms and causes – Mayo Clinic
Amputation is the loss or removal of a body part such as a finger, toe, hand, foot, arm or leg. It can be a life changing experience affecting your ability to move, work, interact with others and maintain your independence. Continuing pain, phantom limb phenomena and emotional trauma can complicate recovery.
Johns Hopkins Medicine: Amputation | Johns Hopkins Medicine
With leg amputee’s, the seating approach can depend on where the amputation is. When in lower extremities, if it is close to the knee or above the knee consideration needs to be given to the cushion & its ability place the leg in the best position for balance & comfort. The center of gravity of the chair will also be affected. With below knee amputations, a separate amputee pad may be clamped onto the wheelchair leg rest. Pressure care must be considered due to the increased weight loading under the torso. With upper extremity amputation, a powerchair may be essential & support added to prevent postural deformities.
Pelvic obliquity is the misalignment of the pelvis, typically where one hip is higher than the other. This condition can lead to abnormal postures due to the spine compressing and becoming misaligned to compensate for any misalignment.
For people with flexible pelvic obliquity, the seat cushion can also play a key role in correcting or accommodating their postural abnormality. If the obliquity is flexible, a wedge is placed under the seat on the low side to level the pelvis. When the Pelvis presents as fixed, we have to be careful we don’t increase the pelvic obliquity. Maybe, a buildup under the high side to accommodate & distribute pressure, but not to the level of the obliquity so there is ability to reduce it over time. Fixed pelvic obliquity doesn’t mean it can’t get worse. Also find the core reason for the pelvic obliquity, is hip pain or subluxation the cause? – These may need to be addressed first.
A pelvic rotation is when an individual presents with one hip further forward in the seat, with one anterior superior iliac spine more forward than the other. This can arise from how an individual sleeps (side lying), dominant side compensatory postures, through to daily activities such as operating a powerchair.
With pelvic rotation, check to see if there is an unaccommodated leg length discrepancy & if there is unaccommodated asymmetry in the individual’s trunk. Sometimes accommodating more wind sweeping can reduce the muscle tone, reduce pelvic rotation & enable a more upright sitting posture.
Posterior pelvic tilt is a condition in which the front of the pelvis rises and the back of the pelvis drops, while the pelvis rotates upwards.
The position of your pelvis matters a great deal to your spinal alignment and your low-back health. Posterior pelvic tilt is caused by an imbalance between the core muscles and the leg muscles. It can cause a variety of uncomfortable symptoms, such as tight hamstrings and back pain.
Verywell Health: Posterior Pelvic Tilt: Causes, Symptoms, and Treatment (verywellhealth.com)
Posterior pelvic Tilt is the most common tendency in wheelchair seating & mostly the cause of the forward migration of the pelvis.
An anterior pelvic tilt is when your pelvis is rotated forward, which forces your spine to curve. It’s often caused by excessive sitting without enough exercise and stretching to counteract the effects of sitting all day. If you have an anterior pelvic tilt, you may notice that the muscles in the front of your pelvis and thighs are tight, while the ones in the back are weak. Your gluteus and abdominal muscles may also be weak.
Healthline: 5 Anterior Pelvic Tilt Exercises (healthline.com)
Sometimes mild anterior tilt of the pelvis is encouraged as it can help to distribute weight through the ischial region & reduce some asymmetries in the spine such as kyphosis, but it also can be the root of substantial lordosis in the spine. Keeping the backrest upright to make better contact with the individual and contouring to allow excess fleshy tissue to protrude below the backrest may help. A mild (5 deg.) degree of seat dump could be tried to reduce the tone.
Tight hamstrings develop when the muscles on the back of the thighs shorten. While the cause of hamstring tightness isn’t always known, it commonly affects athletes as well as people who spend a lot of time sitting, such as students or people who work at desk jobs.
Tight hamstrings can also be a symptom of other conditions, such as a hamstring strain or low back injury.
Verywell Health: Why Hamstrings Get So Tight (and Tips to Loosen Them) (verywellhealth.com)
Unaccommodated tight hamstrings in seating (often accompanied by shortened PSOAS muscles) can pull the pelvis into posterior pelvic. Check the seat depth (too long can increase posterior pelvic migration) & ensure the lower leg can move back sufficiently to relieve the hamstring tension. With windswept postures, accommodating more lateral migration in the seating can reduce the tension on the hamstrings.
The femur is the long bone that connects the hip joint to the knee joint. Anteversion means “leaning forward.” Femoral anteversion is a condition in which the femoral neck leans forward with respect to the rest of the femur. This causes the leg to rotate internally, so that the knee and foot twist toward the midline of the body.
Johns Hopkins Medicine: Femoral Anteversion | Johns Hopkins Medicine
Internal femoral rotation, when severe, can cause hip pain or even the hips to become subluxed. Good medial thigh contouring can help reduce the muscle tone.
Femoral retroversion refers to an abnormal backward rotation of the hip relative to the knee. This condition can affect patients of all ages and leads to abnormal stress in the low back, hip and knee and abnormal gait. Femoral retroversion is a rotational or torsional deformity in which the femur twists backward (outward) relative to the knee.
Set in Motion Physiotherapy: Blog | Set in Motion Physiotherapy (simphysio.com.au)
With paralysis the femurs often externally rotate and may need hip guides to keep in alignment. When there is increased spasticity such as CP with wind sweeping it can be very painful to try to correct this posture. In these cases, going with the body at the beginning, then gradually over time decreasing the level of external rotation may be effective.
Femoral-on-pelvic hip adduction occurs as the femur moves toward, or across, the midline relative to a fixed pelvis. Often found in conjunction with external femoral rotation.
See external femoral rotation.
Hip abduction is the movement of the leg away from the midline of the body. Often found in conjunction with internal femoral rotation.
See internal femoral rotation.
Extensor thrust is an event when nearly all of the muscles in the body contract simultaneously. As a consequence, the person’s body tends to straighten out, as shown in Fig. 1. This motion can injure the occupant, damage the wheelchair, and cause the occupant to slide out of the wheelchair. A danger that the wheelchairs topples over during extensor thrust may be suspected but fortunately, no observations in a clinical environment indicated that extensor thrust led to wheelchairs toppling over. Extensor thrust is a symptom usually occurring in people suffering from brain-related diseases such as cerebral palsy. Extensor thrust occurs because the central nervous system is unable to adequately control or supervise the muscles.
Science Direct: Identification of human-generated forces on wheelchairs during total-body extensor thrusts – ScienceDirect
Firstly, check the occupant can tolerate the angles of the seating system (these may need to be opened or closed to reduce the effect of the muscle tone). Secondly, check for discomfort – pain from a joint, muscle or fleshy tissue can cause the individual to extend.
Flexion is the most common knee deformity in patients with cerebral palsy and frequently occurs in ambulatory children. Flexion at the hip (which causes the leg to lift upwards when lying or the body to lean forwards in standing) Flexion at the knees (causing changes in a person’s standing posture).
Regular monitoring of a user’s range of movement can help to identify flexion contractures at an early stage. Knee flexor contractures may require the footplates to be moved rearwards to accommodate the contracture. Another option may be to reduce the cushion depth to enable the lower leg to move backwards. Tightening of the PSOA muscles may increase posterior pelvic tilt & some accommodation in the backrest may be required if the posture is fixed.
Hypertonia is a medical term for too much muscle tone. It can make arms or legs stiff and difficult to move and often happens when part of the brain or spinal cord is damaged.
National Institute of Neurological Disorders and Stroke:Glossary of Neurological Terms | National Institute of Neurological Disorders and Stroke (nih.gov)
Correction of postures caused by high general tone needs to be undertaken very slowly over a period of time & also in conjunction with other therapies such as physiotherapy, swimming & night-time positioning.
Hypotonia means decreased muscle tone. It can be a condition on its own, called benign congenital hypotonia, or it can be indicative of another problem where there is progressive loss of muscle tone, such as muscular dystrophy or cerebral palsy. It is usually detected during infancy.
Boston Children’s Hospital: Muscle Weakness (Hypotonia) | Boston Children’s Hospital (childrenshospital.org)
Spinal extension & head control are common challenges with underlying low tone. A neutral seat dump (no tilting of the seat base), keeping the backrest more upright (closer to 95 deg.) with a sacral support which allows the scapulars to fall slightly behind the pelvis, stabilizing the spine laterally with external trunk lateral supports, then working on head position. Preventing lateral slippage of the head position can reduce the tendency of the head to fall forwards. Position the upper arms in line with the trunk as these will also help to stabilize the trunk & head.
Ataxia describes poor muscle control that causes clumsy movements. It can affect walking and balance, hand coordination, speech and swallowing, and eye movements.
Ataxia usually results from damage to the part of the brain called the cerebellum or its connections. The cerebellum controls muscle coordination. Many conditions can cause ataxia, including genetic conditions, stroke, tumors, multiple sclerosis, degenerative diseases and alcohol misuse. Certain medicines also can cause ataxia.
Mayo Clinic: Ataxia – Symptoms and causes – Mayo Clinic
Fine tuning to make the wheelchair as efficient for the user as possible, such as wheel height & position, joystick position. A stable base with close support can help to stabilize the individual. Sometimes individuals may benefit from strapping the limbs as this reduces unwanted movement enabling them to concentrate their effort on the task at hand.
The asymmetrical tonic neck reflex (ATNR) is a primitive reflex found in newborn humans that normally vanishes around 6 months of age. Prolonged ATNR can be associated with developmental delays. It becomes hard for them to cross the midline of their bodies, for example, and they can’t handle objects with both hands. In other instances, the child can’t identify which hand or leg to use, causing him to hesitate in movements.
Kyphosis is an excessive forward rounding of the upper back.
In older people, kyphosis is often due to weakness in the spinal bones that causes them to compress or crack. Other types of kyphosis can appear in infants or teens. These types are due to malformation of the spine or wedging of the spinal bones over time.
Mild kyphosis causes few problems. Serious kyphosis can cause pain and be disfiguring. Treatment for kyphosis depends on your age, and the cause and seriousness of the curvature.
Mayo Clinic: Kyphosis – Symptoms and causes – Mayo Clinic
Kyphosis often stems from poor pelvic position & tightening of the hamstrings. First neutralise any seat dump (seat base angle) then concentrate on the pelvic & leg, knees feet position (the foot position can be influential in spinal extension) before attempting the backrest. In the backrest, provide a good sacral block & support under the apex of the kyphosis with adaptable support. The headrest may need to be brought forwards to meet the head to reduce the risk of a lordosis at the neck. Sometimes there is the added challenge of the tendency to fall forward in the seat. Forearm position is critical to spinal extension & head control. Keep the arms positioned beside the trunk (not too forward or too rearward as this can reduce the stability to the trunk). A hip belt & shoulder harness or chest strap can be beneficial to encourage an upright posture.
Lordosis is the medical definition for the forward curved spine in your neck or lower back.
Your cervical spine (the medical name for the part of your spine in your neck) and lumbar spine (your lower back) are naturally curved a little forward, toward the front of your body. This naturally occurring lordosis helps you maintain your posture and absorb shock when you move.
Anything that makes those areas of your spine curve more than they should is called a lordotic curve.
Cleveland Clinic: Lordosis (Swayback): Types, Causes & Symptoms (clevelandclinic.org)
Lordosis of the spine is often accompanied with an anterior tilted pelvis. Position the pelvis first, then stabilize it with the legs, knees & feet before working on the backrest. Check for back pain or insecurity in individual as lordosis can sometimes be a compensatory posture for those. Bring the backrest forward to meet the individuals back (it may need to be more upright to reduce the effect of the Lordosis).
Scoliosis is a sideways curvature of the spine that most often is diagnosed in adolescents. While scoliosis can occur in people with conditions such as cerebral palsy and muscular dystrophy, the cause of most childhood scoliosis is not known.
Most cases of scoliosis are mild, but some curves worsen as children grow. Severe scoliosis can be disabling. An especially serious spinal curve can reduce the amount of space within the chest, making it difficult for the lungs to function properly.
Mayo Clinic: Scoliosis – Symptoms and causes – Mayo Clinic
Overall support starts from a well-positioned pelvis. Then supporting the back where gravity can have the least affect. Due to shortening it may not be possible to reduce a scoliosis but maintain the posture with a backrest which can adapt & support asymmetrical postures and install lateral supports (these may need to be off-set from each other in height to best prop the ribcage). Sometimes tilting the lateral pad is beneficial to support the inside curvature of the trunk and increase the surface contact area.
Instability & fear of falling in a wheelchair can adversely impact posture.
Where there is instability or fear of falling from the wheelchair, lower the seat to floor height, widen the base of support & tighten loose supports to increase the feeling of security to the individual. Increasing the contouring around the user can help with a feeling of stability.
Your Rib-Hip Connection is the distance between your ribcage and your pelvis, or from your lower ribs to your hip bones. Under normal conditions, there is no iliocostal contact due to enough distance between the lower ribs and the iliac crest.
Check the pelvis is positioned well & support by the lower leg, knees & feet. Check there is no hip or pelvic pain on the high side of the pelvic obliquity, as this can be sometimes cause of lifting the hip off the seat. Close lateral support is needed to open the ribcage on the tightened side (this must only be done in conjunction with other physiotherapy such as stretches). Tilting lateral pads may be beneficial to support on the underside of the internal curvature.
Complete dislocations (luxation): A complete dislocation happens when the bones in your joint are totally separated and pushed out of place.
Subluxation: Subluxation is the medical term for a partial dislocation. You have a subluxation if something pulls your joint apart and the bones still touch, just not as completely as usual.
Cleveland Clinic: Dislocation: Types, Treatment & Prevention (clevelandclinic.org)
Subluxation & dislocation can be very painful & often can cause compensatory sitting postures. It is best practice to address these issues first with physiotherapy or surgery before attempting to adjust the seating.
Spinal fusion is surgery to connect two or more bones in any part of the spine. Connecting them prevents movement between them. Preventing movement helps to prevent pain.
During spinal fusion, a surgeon places bone or a bonelike material in the space between two spinal bones. Metal plates, screws or rods might hold the bones together. They then can fuse and heal as one bone.
Mayo Clinic: Spinal fusion – Mayo Clinic
It is generally recommended to wait until after surgery before attempting to seat an individual who is waiting for spinal surgery. Often the result of the surgery will straighten the back & in turn lengthen the trunk. Asymmetrical contouring may be required to be reduced or placed at a different position in the back than before surgery.
Spasticity is a disruption in muscle movement patterns that causes certain muscles to contract all at once when you try to move or even at rest. The muscles remain contracted and resist being stretched. It interferes with movement and can also affect your speech and gait (walk).
Spasticity can vary greatly in how it affects someone — it may be as mild as the feeling of tightness of your muscles or may be so severe that it causes painful, uncontrollable stiffness and spasms of your extremities.
Cleveland Clinic: Spasticity: What It Is, Causes, Symptoms & Treatment (clevelandclinic.org)
When there is increased spasticity the risk of abnormal asymmetries to the body structure increases due to unbalanced muscle groups. Therapy programmes should be considered including stretches, exercises for coordination & flexibility, swimming and sleep positioning systems.
Near drowning occurs when water enters your lungs, and you stop breathing. Your brain does not get the oxygen it needs, and major organ systems may begin to shut down.
The more severe the case due to hypoxia, the more difficult these patients are to seat. The patient may present with rigid muscles & require constant breathing aids & sometimes cooling aids. Some of these individuals can be very difficult to seat in an upright position & compromises may have to be made.