Diagnosis To Device

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  • Spastic CP - hemiplegia

    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 CP - Diplegia

    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).

  • Spastic CP - Quadriplegia

    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.

  • Dyskinetic CP (athetoid or Choreoathetoid)

    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.

  • Ataxic CP

    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 CP

    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.

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  • Stroke - Hemmorrhagi

    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.

  • Stroke - Transient Ischemic Attack (mini stroke)

    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)

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  • Sclerosis

    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.

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  • Duchenne

    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

    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

    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

    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)

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  • Meningocele

    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.

  • Myelomeningocele

    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)

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  • Epilepsy

    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.

  • Seizures

    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

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  • Amputee

    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.

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  • Pelvic Obliquity

    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.

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  • Pelvic Rotation

    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.

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  • Flexion

    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.

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  • ATNR (Asymmetrical Tonal Neck Reflex)

    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.

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