Diseases » Spinocerebellar Ataxia » Causes

Causes of Spinocerebellar Ataxia

Spinocerebellar Ataxia Causes: Book Excerpts

• Differential Diagnosis - Ataxia
• Differential Diagnosis - Ataxia
• Medical causes - Ataxia
• Medical causes - Ataxia
• Differential Overview - Ataxia
• Medical causes - Ataxia
• Medical causes - Ataxia
• Principal Causes of Ataxia - Ataxia
• Medical causes - Ataxia

Spinocerebellar Ataxia as a symptom:

Conditions listing Spinocerebellar Ataxia as a symptom may also be potential underlying causes of Spinocerebellar Ataxia. Our database lists the following as having Spinocerebellar Ataxia as a symptom of that condition:

• Ataxia - hypogonadism - choroidal dystrophy
• Boucher-Neuhauser syndrome
• Spinocerebellar ataxia 4

Related information on causes of Spinocerebellar Ataxia:

As with all medical conditions, there may be many causal factors. Further relevant information on causes of Spinocerebellar Ataxia may be found in:

• Genetics of Spinocerebellar Ataxia
• Hidden causes of Spinocerebellar Ataxia

Causes of Spinocerebellar Ataxia: Online Medical Books

16 MEDICAL BOOKS ONLINE! Review excerpts from medical books online, free, without registration, for more information about the causes of Spinocerebellar Ataxia.

Ataxia: Differential Diagnosis
(In a Page: Signs and Symptoms)

• Orthopedic issues affecting the foot, ankle, leg, knee, or hip

• Peripheral neuropathy (sensory and/or motor)
  –Slapping gait: Sensory neuropathies may result in a tendency to slap the feet firmly against the ground to improve proprioceptive input
  –Steppage gait: Seen in patients with foot drop
  –The classic tabetic gait combines both stepping and slapping gaits
• Mononeuropathy/radiculopathy affecting the lower extremities may result in gait abnormalities (e.g., either a peroneal neuropathy or L5 radiculopathy can cause a unilateral steppage gait)
• Myelopathy
  –Patients with bilateral lower extremity weakness and hypertonicity secondary to a spinal cord lesion may exhibit a spastic gait with stiffness of both legs and a tendency toward scissoring of the legs with walking
• Brainstem or cortical lesions (e.g., multiple sclerosis, CVA)
  –Most commonly result in a hemiparetic gait with circumduction of the weak leg
• Cerebellar lesions
  –Result in an ataxic gait, which tends to be wide-based, irregular, and staggering
• Intoxications
• Parkinsonism
  –Patients exhibit stooped posture, decreased arm swing, and shuffling (take many small steps)
• Myopathies
  –Tend to produce a waddling gait because of weakness of the trunk, hip, and proximal lower extremity muscles
• Spinocerebellar ataxia
• Hereditary spastic paraparesis
• Hysterical gaits
• Inherited neuropathies (e.g., Charcot-Marie-Tooth disease)
• GALOP syndrome (gait disorder, autoantibodies, late age onset, polyneuropathy)
• Normal pressure hydrocephalus
• Infection (e.g., neurosyphillis, meningitis)
• Vitamin B₁₂ or thiamine deficiency

» READ BOOK EXCERPT ONLINE »

Source: In a Page: Signs and Symptoms, 2004

Ataxia: Differential Diagnosis
(In A Page: Pediatric Signs and Symptoms)

• Infectious
  –Postinfectious cerebellitis: Due to varicella, EBV, mumps, Legionella, hepatitis A
  –Encephalitis, acute disseminated encephalomyelitis (ADEM)
  –Cerebellar abscess
  –Labyrinthitis
• Toxic/metabolic encephalopathy
  –Phenytoin, carbamazepine, antihistamines, sedatives, and ethanol
• Posterior fossa tumor
  –Medulloblastoma, glioma
• Opsoclonus/myoclonus syndrome: May be postinfectious or paraneoplastic (reaction most likely to neuroblastoma)
• Friedreich ataxia: Absent DTRs, positive Babinski sign, proprioceptive sensory loss, dysarthria, cardiomyopathy, diabetes
• Ataxia-telangiectasia
• Miller-Fisher variant of Guillain-Barré syndrome, with areflexia, ophthalmoparesis
• Basilar migraine
• Postconcusssion
• Seizure
• Arnold-Chiari, Dandy-Walker malformation
• Cerebellar hemorrhage or ischemia
• Multiple sclerosis
• Vitamin E (AVED), biotinidase deficiency
• Metabolic disorders
  –Urea cycle disorders
  –Organic acidurias (maple syrup urine disease)
  –Carbohydrate-deficient glycoprotein syndrome
• Sphingolipidoses (GM2, Niemann-Pick type C, metachromatic leukodystrophy, Krabbe disease)
• Neuronal ceroid-lipofuscinosis
• Mitochondrial disease
  –NARP, MERRF, Kearns-Sayre syndrome
• Spinocerebellar ataxias (SCA)
• Hartnup disease
• Refsum disease
• Episodic ataxia type I/II (channelopathy)
• Celiac disease
  –Have cerebellar calcification
• Conversion disorder

» READ BOOK EXCERPT ONLINE »

Source: In A Page: Pediatric Signs and Symptoms, 2007

Ataxia: Medical causes
(Handbook of Signs & Symptoms (Third Edition))

❑ Cerebellar abscess. Cerebellar abscess commonly causes limb ataxia on the same side as the lesion as well as gait and truncal ataxia. Typically, the initial symptom is a headache localized behind the ear or in the occipital region, followed by oculomotor palsy, fever, vomiting, an altered LOC, and coma.

❑ Cerebellar hemorrhage. With cerebellar hemorrhage, a life-threatening disorder, ataxia is usually acute but transient. Unilateral or bilateral ataxia affects the trunk, gait, or limbs. The patient initially experiences repeated vomiting, occipital headache, vertigo, oculomotor palsy, dysphagia, and dysarthria. Later signs, such as a decreased LOC or coma, signal impending herniation.

❑ Creutzfeldt-Jakob disease. Creutz-feldt-Jakob disease is a rapidly progressive dementia accompanied by neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, visual disturbances, and paralysis. It generally affects adults ages 40 to 65.

❑ Diabetic neuropathy. Peripheral nerve damage due to diabetes mellitus may cause sensory ataxia, extremity pain, slight leg weakness, skin changes, and bowel and bladder dysfunction.

❑ Diphtheria. Within 4 to 8 weeks of the onset of symptoms, a life-threatening neuropathy can produce sensory ataxia. Diphtheria can be accompanied by fever, paresthesia, and paralysis of the limbs and, sometimes, the respiratory muscles.

❑ Encephalomyelitis. Encephalomyelitis a complication of measles, smallpox, chickenpox, or rubella or of rabies or smallpox vaccination that may damage cerebrospinal white matter. Rarely, it’s accompanied by cerebellar ataxia. Other signs and symptoms include headache, fever, vomiting, an altered LOC, paralysis, seizures, oculomotor palsy, and pupillary changes.

❑ Friedreich’s ataxia. A progressive familial disorder, Friedreich’s ataxia affects the spinal cord and cerebellum. It causes gait ataxia, followed by truncal, limb, and speech ataxia. Other signs and symptoms include pes cavus, kyphoscoliosis, cranial nerve palsy, and motor and sensory deficits. A positive Babinski’s reflex may appear.

❑ Guillain-Barré syndrome. Peripheral nerve involvement usually follows a mild viral infection, rarely leading to sensory ataxia. Guillain-Barré syndrome also causes ascending paralysis and possibly respiratory distress.

❑ Hepatocerebral degeneration. Patients who survive hepatic coma are occasionally left with residual neurologic defects, including mild cerebellar ataxia with a wide-based, unsteady gait. Ataxia may be accompanied by an altered LOC, dysarthria, rhythmic arm tremors, and choreoathetosis of the face, neck, and shoulders.

❑ Multiple sclerosis (MS). Nystagmus and cerebellar ataxia commonly occur in MS, but they aren’t always accompanied by limb weakness and spasticity. Speech ataxia (especially scanning) may occur as well as sensory ataxia from spinal cord involvement. During remissions, ataxia may subside or may even disappear. During exacerbations, it may reappear, worsen, or even become permanent. MS also causes optic neuritis, optic atrophy, numbness and weakness, diplopia, dizziness, and bladder dysfunction.

❑ Olivopontocerebellar atrophy. Olivopontocerebellar atrophy produces gait ataxia and, later, limb and speech ataxia. Rarely, it produces an intention tremor. It’s accompanied by choreiform movements, dysphagia, and loss of sphincter tone.

❑ Poisoning. Chronic arsenic poisoning may cause sensory ataxia, along with headache, seizures, an altered LOC, motor deficits, and muscle aching. Chronic mercury poisoning causes gait and limb ataxia, principally of the arms. It also causes tremors of the extremities, tongue, and lips; mental confusion; mood changes; and dysarthria.

❑ Polyneuropathy. Carcinomatous and myelomatous polyneuropathy may occur before detection of the primary tumor in cancer, multiple myeloma, or Hodgkin’s disease. Signs and symptoms include ataxia, severe motor weakness, muscle atrophy, and sensory loss in the limbs. Pain and skin changes may also occur.

❑ Porphyria. Porphyria affects the sensory and, more frequently, the motor nerves, possibly leading to ataxia. It also causes abdominal pain, mental disturbances, vomiting, headache, focal neurologic defects, an altered LOC, generalized seizures, and skin lesions.

❑ Posterior fossa tumor. Gait, truncal, or limb ataxia is an early sign and may worsen as the tumor enlarges. It’s accompanied by vomiting, headache, papilledema, vertigo, oculomotor palsy, a decreased LOC, and motor and sensory impairments on the same side as the lesion.

❑ Spinocerebellar ataxia. With spinocerebellar ataxia, the patient may initially experience fatigue, followed by stiff-legged gait ataxia. Eventually, limb ataxia, dysarthria, static tremor, nystagmus, cramps, paresthesia, and sensory deficits occur.

❑ Stroke. In stroke, occlusions in the vertebrobasilar arteries halt blood flow to cause infarction in the medulla, pons, or cerebellum that may lead to ataxia. Ataxia may occur at the onset of stroke and remain as a residual deficit. Worsening ataxia during the acute phase may indicate extension of the stroke or severe swelling. Ataxia may be accompanied by unilateral or bilateral motor weakness, a possible altered LOC, sensory loss, vertigo, nausea, vomiting, oculomotor palsy, and dysphagia.

❑ Wernicke’s disease.The result of thiamine deficiency, Wernicke’s disease produces gait ataxia and, rarely, intention tremor or speech ataxia. With severe ataxia, the patient may be unable to stand or walk. Ataxia decreases with thiamine therapy. Associated signs and symptoms include nystagmus, diplopia, ocular palsies, confusion, tachycardia, exertional dyspnea, and orthostatic hypotension.

Other causes

❑ Drugs. Toxic levels of anticonvulsants, especially phenytoin, may result in gait ataxia. Toxic levels of anticholinergics and tricyclic antidepressants may also result in ataxia. Aminoglutethimide causes ataxia in about 10% of patients; however, this effect usually disappears 4 to 6 weeks after drug therapy is discontinued.

» READ BOOK EXCERPT ONLINE »

Source: Handbook of Signs & Symptoms (Third Edition), 2006

Ataxia: Medical causes
(Professional Guide to Signs & Symptoms (Fifth Edition))

Cerebellar abscess

Cerebellar abscess commonly causes limb ataxia on the same side as the lesion as well as gait and truncal ataxia. Typically, the initial symptom is headache localized behind the ear or in the occipital region, followed by oculomotor palsy, fever, vomiting, altered LOC, and coma.

Cerebellar hemorrhage

Cerebellar hemorrhage is a life-threatening disorder in which ataxia is usually acute but transient. Unilateral or bilateral ataxia affects the trunk, gait, or limbs. The patient initially experiences repeated vomiting, an occipital headache, vertigo, oculomotor palsy, dysphagia, and dysarthria. Later signs, such as decreased LOC or coma, signal impending herniation.

Cranial trauma

Cranial trauma rarely produces ataxia, but when it does, the ataxia is usually unilateral; bilateral ataxia suggests traumatic hemorrhage. Associated signs and symptoms include vomiting, headache, decreased LOC, irritability, and focal neurologic defects. If the cerebral hemispheres are also affected, focal or generalized seizures may occur.

Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is a rapidly progressive dementia accompanied by neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, visual disturbances, and paralysis. It generally affects adults ages 40 to 65.

Diabetic neuropathy

Peripheral nerve damage due to diabetes mellitus may cause sensory ataxia, extremity pain, slight leg weakness, skin changes, and bowel and bladder dysfunction.

Diphtheria

Within 4 to 8 weeks of the onset of symptoms, a life-threatening neuropathy can produce sensory ataxia. Diphtheria can be accompanied by fever, paresthesia, and paralysis of the limbs and possibly the respiratory muscles.

Encephalomyelitis

Encephalomyelitis is a complication of measles, smallpox, chickenpox, or rubella or of rabies or smallpox vaccine that may damage cerebrospinal white matter. Rarely, it’s accompanied by cerebellar ataxia. Other signs and symptoms include headache, fever, vomiting, altered LOC, paralysis, seizures, oculomotor palsy, and pupillary changes.

Friedreich’s ataxia

Friedreich’s ataxia is a progressive familial disorder that affects the spinal cord and cerebellum. It causes gait ataxia, followed by truncal, limb, and speech ataxia. Other signs and symptoms include pes cavus, kyphoscoliosis, cranial nerve palsy, and motor and sensory deficits. A positive Babinski’s reflex may appear.

Guillain-Barré syndrome

This syndrome usually begins with a mild viral infection, followed by peripheral nerve involvement and, rarely, sensory ataxia. It may also cause ascending paralysis and respiratory distress.

Hepatocerebral degeneration

Some patients who survive hepatic coma are left with residual neurologic defects, including mild cerebellar ataxia with a wide-based, unsteady gait. Ataxia may be accompanied by altered LOC, dysarthria, rhythmic arm tremors, and choreoathetosis of the face, neck, and shoulders.

Hyperthermia

Cerebellar ataxia occurs if the patient survives the coma and seizures characteristic of the acute phase of hyperthermia. Subsequent findings include spastic paralysis, dementia, and slowly resolving confusion.

Metastatic cancer

Cancer that metastasizes to the cerebellum may cause gait ataxia accompanied by headache, dizziness, nystagmus, decreased LOC, nausea, and vomiting.

Multiple sclerosis (MS)

Nystagmus and cerebellar ataxia commonly occur in MS, but they aren’t always accompanied by limb weakness and spasticity. The patient may also have speech ataxia (especially scanning) as well as sensory ataxia from spinal cord involvement. During remissions, ataxia may subside or even disappear. During exacerbations, it may reappear, worsen, or even become permanent. MS also causes optic neuritis, optic atrophy, numbness and weakness, diplopia, dizziness, and bladder dysfunction.

Olivopontocerebellar atrophy

Olivopontocerebellar atrophy produces gait ataxia and, later, limb and speech ataxia. Rarely, it produces an intention tremor. It’s accompanied by choreiform movements, dysphagia, and loss of sphincter tone.

Polyarteritis nodosa

Acute or subacute polyarteritis may cause sensory ataxia, abdominal and limb pain, hematuria, fever, and elevated blood pressure.

Polyneuropathy

Carcinomatous and myelomatous polyneuropathy may occur before detection of the primary tumor in cancer, multiple myeloma, or Hodgkin’s disease. Signs and symptoms include ataxia, severe motor weakness, muscle atrophy, and sensory loss in the limbs. Pain and skin changes may also occur.

Porphyria

Porphyria affects the sensory and, more commonly, the motor nerves, possibly leading to ataxia. It also causes abdominal pain, mental disturbances, vomiting, headache, focal neurologic defects, altered LOC, generalized seizures, and skin lesions.

Posterior fossa tumor

Gait, truncal, or limb ataxia is an early sign and may worsen as the tumor enlarges. It’s accompanied by vomiting, headache, papilledema, vertigo, oculomotor palsy, decreased LOC, and motor and sensory impairment on the same side as the lesion.

Spinocerebellar ataxia

In spinocerebellar ataxia, the patient may initially experience fatigue, followed by stiff-legged gait ataxia. Eventually, limb ataxia, dysarthria, static tremor, nystagmus, cramps, paresthesia, and sensory deficits occur.

Stroke

In a stroke, occlusions in the vertebrobasilar arteries halt blood flow, causing infarction in the medulla, pons, or cerebellum that may lead to ataxia. Ataxia may occur at the onset of the stroke and remain as a residual deficit. Worsening ataxia during the acute phase may indicate extension of the stroke or severe swelling. Ataxia may be accompanied by unilateral or bilateral motor weakness, altered LOC, sensory loss, vertigo, nausea, vomiting, oculomotor palsy, and dysphagia.

Syringomyelia

Syringomyelia is a chronic degenerative disorder that may cause a mixed spastic-ataxic gait. It’s associated with loss of pain and temperature sensation (but preservation of touch sensation), skin changes, amyotrophy, and thoracic scoliosis.

Wernicke’s encephalopathy

The result of a thiamine deficiency, Wernicke’s encephalopathy produces gait ataxia and, rarely, intention tremor or speech ataxia. With severe ataxia, the patient may be unable to stand or walk. Ataxia decreases with thiamine therapy. Associated signs and symptoms include nystagmus, diplopia, ocular palsies, confusion, tachycardia, exertional dyspnea, and orthostatic hypotension.

Other causes

Drugs

Toxic levels of anticonvulsants, especially phenytoin, may result in gait ataxia. Toxic levels of anticholinergics and tricyclic antidepressants may also result in ataxia. Aminoglutethimide causes ataxia in about 10% of patients; however, this effect usually disappears 4 to 6 weeks after drug therapy is discontinued.

Poisoning

Chronic arsenic poisoning may cause sensory ataxia along with headache, seizures, altered LOC, motor deficits, and muscle aching. Chronic mercury poisoning causes gait ataxia and limb ataxia, principally of the arms. Chronic mercury poisoning also causes tremors of the extremities, tongue, and lips; mental confusion; mood changes; and dysarthria.

» READ BOOK EXCERPT ONLINE »

Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006

Ataxia: Differential Overview
(Field Guide to Bedside Diagnosis)

Chronic

❑ Vitamin B₁₂ deficiency

❑ Parkinsonism

❑ Myopathy

❑ Cervical spondylosis

❑ Multiple sclerosis

❑ Multiple subcortical strokes

❑ Alcoholic cerebellar degeneration

❑ Hydrocephalus

❑ Frontal lobe tumor

❑ Cerebellar tumor

❑ Spinocerebellar degeneration

❑ Syringomyelia

❑ Tabes dorsalis

❑ Chorea

Acute

❑ Alcohol intoxication

❑ Labyrinthitis

❑ Cerebellar hemorrhage

❑ Cerebellar infarct

❑ Guillain-Barré syndrome

❑ Hysterical

❑ Parietal apraxia

» READ BOOK EXCERPT ONLINE »

Source: Field Guide to Bedside Diagnosis, 2007

Ataxia: Medical causes
(Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series)

Cerebellar abscess

Cerebellar abscess commonly causes limb ataxia on the same side as the lesion as well as gait and truncal ataxia. Typically, the initial symptom is headache localized behind the ear or in the occipital region, followed by oculomotor palsy, fever, vomiting, altered LOC, and coma.

Cerebellar hemorrhage

With cerebellar hemorrhage, a life-threatening disorder, ataxia is usually acute but transient. Unilateral or bilateral ataxia affects the trunk, gait, or limbs. The patient initially experiences repeated vomiting, occipital headache, vertigo, oculomotor palsy, dysphagia, and dysarthria. Later signs, such as decreased LOC or coma, signal impending herniation.

Cranial trauma

Ataxia is rare with cranial trauma; if it occurs, it’s typically unilateral. Bilateral ataxia suggests traumatic hemorrhage. Associated signs and symptoms include vomiting, headache, decreased LOC, irritability, and focal neurologic defects. If the cerebral hemispheres are also affected, focal or generalized seizures may occur.

Creutzfeldt-Jakob disease

Creutzfeldt-Jacob disease is a rapidly progressive dementia accompanied by neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, vision disturbances, and paralysis.

Diabetic neuropathy

Peripheral nerve damage due to diabetes mellitus may cause sensory ataxia, extremity pain, slight leg weakness, skin changes, and bowel and bladder dysfunction.

Diphtheria

Within 4 to 8 weeks of the onset of diphtheria symptoms, a life-threatening neuropathy develops, possibly producing sensory ataxia and paralysis of respiratory muscles. Diphtheria can be accompanied by fever, paresthesia, and limb paralysis and, sometimes, respiratory muscle paralysis.

Encephalomyelitis

Encephalomyelitis can, rarely, be accompanied by cerebellar ataxia. Encephalomyelitis, which may lead to damage of the cerebrospinal white matter, is a complication associated with measles, smallpox, chickenpox, or rubella. It may also occur due to rabies or smallpox vaccinations. Signs and symptoms of encephalomyelitis include headache, fever, vomiting, altered LOC, paralysis, seizures, oculomotor palsy, and pupillary changes.

Friedreich’s ataxia

Friedreich’s ataxia, a progressive familial disorder, affects the spinal cord and cerebellum. It causes gait ataxia initially, followed by truncal, limb, and speech ataxia. Other signs and symptoms include pes cavus, kyphoscoliosis, cranial nerve palsy, and motor and sensory deficits. A positive Babinski’s response may appear.

Guillain-Barré syndrome

Peripheral nerve involvement usually follows a mild viral infection and may, rarely, lead to sensory ataxia. Guillain-Barré syndrome also causes ascending paralysis and may lead to respiratory distress.

Hepatocerebral degeneration

Residual neurologic defects, including mild cerebellar ataxia with a wide-based, unsteady gait, occasionally remain following recovery from hepatic coma. Ataxia may be accompanied by altered LOC, dysarthria, rhythmic arm tremors, and choreoathetosis of the face, neck, and shoulders.

Hyperthermia

Cerebellar ataxia can occur in patients who survive the coma and seizures characteristic of the acute phase of hyperthermia. Subsequent findings include spastic paralysis, dementia, and slowly resolving confusion.

Metastatic cancer

Cancer that metastasizes to the cerebellum may cause gait ataxia accompanied by headache, dizziness, nystagmus, decreased LOC, nausea, and vomiting.

Multiple sclerosis (MS)

Nystagmus and cerebellar ataxia commonly occur in MS; limb weakness and spasticity may occur as well. Speech ataxia (especially scanning) may occur, and spinal cord involvement may result in sensory ataxia. During remissions, ataxia may subside or even disappear. During exacerbations, it may reappear, worsen, or become permanent. MS also causes optic neuritis, optic atrophy, numbness and weakness, diplopia, dizziness, and bladder dysfunction.

Olivopontocerebellar atrophy

Olivopontocerebellar atrophy produces gait ataxia and, later, limb and speech ataxia. In rare instances, it produces an intention tremor. It’s accompanied by choreiform movements, dysphagia, and loss of sphincter tone.

Poisoning

Chronic arsenic poisoning may cause sensory ataxia, along with headache, seizures, altered LOC, motor deficits, and muscle aching. Chronic mercury poisoning causes gait and limb ataxia, principally of the arms. It also causes mental confusion, mood changes, dysarthria, and tremors of the extremities, tongue, and lips.

Polyarteritis nodosa

Acute or subacute polyarteritis may cause sensory ataxia, abdominal and limb pain, hematuria, fever, and elevated blood pressure.

Polyneuropathy

Carcinomatous and myelomatous polyneuropathy may occur before detection of the primary tumor in cancer, multiple myeloma, or Hodgkin’s disease. Signs and symptoms include ataxia, severe motor weakness, muscle atrophy, and sensory loss in the limbs. Pain and skin changes may also occur.

Porphyria

Porphyria affects the sensory and, more commonly, the motor nerves, possibly leading to ataxia. It also causes abdominal pain, mental disturbances, vomiting, headache, focal neurologic defects, altered LOC, generalized seizures, and skin lesions.

Posterior fossa tumor

Gait, truncal, or limb ataxia may occur early and worsen as the tumor enlarges. Other signs and symptoms include vomiting, headache, papilledema, vertigo, oculomotor palsy, decreased LOC, and motor and sensory impairments on the same side as the lesion.

Spinocerebellar ataxia

With spinocerebellar ataxia, the patient may initially experience fatigue, followed by stiff-legged gait ataxia. Eventually, limb ataxia, dysarthria, static tremor, nystagmus, cramps, paresthesia, and sensory deficits occur.

Stroke

Occlusions in the vertebrobasilar arteries halt blood flow, leading to infarction in the medulla, pons, or cerebellum. Ataxia may occur at the onset of stroke and remain as a residual deficit. Worsening ataxia during the acute phase may indicate extension of the stroke or severe swelling. Ataxia may be accompanied by unilateral or bilateral motor weakness, possible altered LOC, sensory loss, vertigo, nausea, vomiting, oculomotor palsy, and dysphagia.

Syringomyelia

Syringomyelia, a chronic, degenerative disorder, may cause a mixed spastic-ataxic gait. It’s associated with loss of pain and temperature sensations (but preservation of touch sensation), skin changes, amyotrophy, and thoracic scoliosis.

Wernicke’s disease

The result of thiamine deficiency, Wernicke’s disease produces gait ataxia and, rarely, intention tremor or speech ataxia. With severe ataxia, the patient may be unable to stand or walk. Ataxia decreases with thiamine therapy. Associated signs and symptoms include nystagmus, diplopia, ocular palsies, confusion, tachycardia, exertional dyspnea, and orthostatic hypotension.

Other causes

Drugs

Toxic levels of anticonvulsants, especially phenytoin, may result in gait ataxia. Toxic levels of anticholinergics and tricyclic antidepressants may also result in ataxia. Aminoglutethimide causes ataxia in about 10% of patients; however, this effect usually disappears 4 to 6 weeks after drug therapy is discontinued.

» READ BOOK EXCERPT ONLINE »

Source: Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series, 2007

Ataxia: Medical causes
(Signs & Symptoms: A 2-in-1 Reference for Nurses)

Cerebellar abscess

Cerebellar abscess commonly causes limb ataxia on the same side as the lesion as well as gait and truncal ataxia. Typically, the initial symptom is headache localized behind the ear or in the occipital region, followed by oculomotor palsy, fever, vomiting, altered LOC, and coma.

Cerebellar hemorrhage

With cerebellar hemorrhage, a life-threatening disorder, ataxia is usually acute but transient. Unilateral or bilateral ataxia affects the trunk, gait, or limbs. The patient initially experiences repeated vomiting, occipital headache, vertigo, oculomotor palsy, dysphagia, and dysarthria. Later signs, such as decreased LOC or coma, signal impending herniation.

Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is a rapidly progressive dementia accompanied by neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, vision disturbances, and paralysis. It generally affects adults ages 40 to 65.

Diabetic neuropathy

Peripheral nerve damage due to diabetes mellitus may cause sensory ataxia, extremity pain, slight leg weakness, skin changes, and bowel and bladder dysfunction. As neuropathy progresses, the patient may report numbness in the feet. Reduced proprioception and sensation may produce an unsteady gait.

Diphtheria

Within 4 to 8 weeks of the onset of symptoms of diphtheria, a life-threatening neuropathy can produce sensory ataxia. Diphtheria can be accompanied by fever, paresthesia, and paralysis of the limbs and, sometimes, the respiratory muscles.

Hepatocerebral degeneration

Patients who survive hepatic coma are occasionally left with residual neurologic defects, including mild cerebellar ataxia with a wide-based, unsteady gait. Ataxia may be accompanied by altered LOC, dysarthria, rhythmic arm tremors, and choreoathetosis of the face, neck, and shoulders.

Hyperthermia

With hyperthermia, cerebellar ataxia occurs if the patient survives the coma and seizures characteristic of the acute phase. Subsequent findings include spastic paralysis, dementia, and slowly resolving confusion.

Metastatic cancer

Cancer that metastasizes to the cerebellum may cause gait ataxia accompanied by headache, dizziness, nystagmus, decreased LOC, nausea, and vomiting. A cerebellar tumor may produce gait ataxia, dizziness, muscle incoordination, and nystagmus. The patient may fall toward the side with the lesion.

Multiple sclerosis

Nystagmus and cerebellar ataxia commonly occur in multiple sclerosis, but they aren’t always accompanied by limb weakness and spasticity. Speech ataxia (especially scanning) may occur as well as sensory ataxia from spinal cord involvement. During remissions, ataxia may subside or even disappear. During exacerbations, it may reappear, worsen, or even become permanent. Multiple sclerosis also causes optic neuritis, optic atrophy, numbness and weakness, diplopia, dizziness, and bladder dysfunction.

Poisoning

Chronic arsenic poisoning may cause sensory ataxia, along with headache, seizures, altered LOC, motor deficits, and muscle aching. Chronic mercury poisoning causes gait ataxia and limb ataxia, principally of the arms. It also causes tremors of the extremities, tongue, and lips; mental confusion; mood changes; and dysarthria.

Polyarteritis nodosa

Acute or subacute polyarteritis nodosa may cause sensory ataxia, abdominal and limb pain, hematuria, fever, and elevated blood pressure. Other findings include myalgia, headache, joint pain, and weakness.

Polyneuropathy

Carcinomatous and myelomatous polyneuropathy may occur before detection of the primary tumor in cancer, multiple myeloma, or Hodgkin’s disease. Signs and symptoms include ataxia, severe motor weakness, muscle atrophy, and sensory loss in the limbs. Pain and skin changes may also occur.

Posterior fossa tumor

Gait, truncal, or limb ataxia is an early sign of a posterior fossa tumor and may worsen as the tumor enlarges. It’s accompanied by vomiting, headache, papilledema, vertigo, oculomotor palsy, decreased LOC, and motor and sensory impairments on the same side as the lesion.

Spinocerebellar ataxia

With spinocerebellar ataxia, the patient may initially experience fatigue, followed by stiff-legged gait ataxia. Eventually, limb ataxia, dysarthria, static tremor, nystagmus, cramps, paresthesia, and sensory deficits occur.

Stroke

With stroke, occlusions in the vertebrobasilar arteries halt blood flow to cause infarction in the medulla, pons, or cerebellum that may lead to ataxia. Ataxia may occur at the onset of stroke and remain as a residual deficit. Worsening ataxia during the acute phase may indicate extension of the stroke or severe swelling. Ataxia may be accompanied by unilateral or bilateral motor weakness, possible altered LOC, sensory loss, vertigo, nausea, vomiting, oculomotor palsy, and dysphagia.

Wernicke’s disease

The result of thiamine deficiency, Wernicke’s disease produces gait ataxia and, rarely, intention tremor or speech ataxia. With severe ataxia, the patient may be unable to stand or walk. Ataxia decreases with thiamine therapy. Associated signs and symptoms include nystagmus, diplopia, ocular palsies, confusion, tachycardia, exertional dyspnea, and orthostatic hypotension.

Other causes

Drugs

Toxic levels of anticonvulsants, especially phenytoin, may result in gait ataxia. Toxic levels of anticholinergics and tricyclic antidepressants may also result in ataxia. Aminoglutethimide causes ataxia in about 10% of patients; however, this effect usually disappears 4 to 6 weeks after drug therapy is discontinued.

» READ BOOK EXCERPT ONLINE »

Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007

Ataxia: Principal Causes of Ataxia
(The Diagnostic Approach to Symptoms and Signs in Pediatrics)

1. Acuteataxia
   1. Drugs,chemicals, and toxins
   2. Head trauma
   3. Infection/inflammation
      1. Meningitis
      2. Encephalitis
      3. Labyrinthitis
      4. Brain abscess
   4. Brain tumor
   5. Hydrocephalus
   6. Postinfectious/immune disorders
      1. Acutecerebellar ataxia of childhood
      2. Acute disseminated encephalomyelitis
      3. Miller Fisher syndrome
      4. Multiple sclerosis
      5. Myoclonic encephalopathy and neuroblastoma
   7. Vascular malformation
   8. Conversion reaction
2. Episodic ataxia
   1. Epilepsy
   2. Basilar artery migraine
   3. Benign paroxysmal vertigo
   4. Metabolic disorders
      1. Maplesyrup urine disease (intermittent)
      2. Hartnup disease
      3. Pyruvate dehydrogenase complex deficiency
   5. Dominant recurrent ataxias
3. Chronic or progressive ataxia
   1. Braintumor
   2. Hydrocephalus
   3. Multiple sclerosis
   4. Congenital malformations of the cerebellumand/or brainstem
   5. Cerebral palsy
   6. Previous brain insults
   7. Spinocerebellar degeneration disorders
      1. Friedreichataxia
      2. Spinocerebellar degenerations
   8. Ataxia telangiectasia
   9. Metabolic disorders
      1. Refsumdisease
      2. Urea cycle defects (argininosuccinicaciduria)
      3. Respiratory chain disorders
      4. Abetalipoproteinemia

» READ BOOK EXCERPT ONLINE »

Source: The Diagnostic Approach to Symptoms and Signs in Pediatrics, 2006

Ataxia: Medical causes
(Nursing: Interpreting Signs and Symptoms)

Cerebellar abscess.Cerebellar abscess commonly causes limb ataxia on the same side as the lesion as well as gait and truncal ataxia. Typically, the initial symptom is a headache localized behind the ear or in the occipital region, followed by oculomotor palsy, fever, vomiting, an altered LOC, and coma.

Cerebellar hemorrhage.With cerebellar hemorrhage, a life-threatening disorder, ataxia is usually acute but transient. Unilateral or bilateral ataxia affects the trunk, gait, or limbs. The patient initially experiences repeated vomiting, occipital headache, vertigo, oculomotor palsy, dysphagia, and dysarthria. Later signs, such as a decreased LOC or coma, signal impending herniation.

Creutzfeldt-Jakob disease.Creutzfeldt-Jakob disease is a rapidly progressive dementia accompanied by neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, visual disturbances, and paralysis. It generally affects adults ages 40 to 65.

Diabetic neuropathy.Peripheral nerve damage due to diabetes mellitus may cause sensory ataxia, extremity pain, slight leg weakness, skin changes, and bowel and bladder dysfunction.

Diphtheria.Within 4 to 8 weeks of the onset of symptoms, a life-threatening neuropathy can produce sensory ataxia. Diphtheria can be accompanied by fever, paresthesia, and paralysis of the limbs and, sometimes, the respiratory muscles.

Encephalomyelitis.Encephalomyelitis is a complication of measles, smallpox, chickenpox, or rubella or of rabies or smallpox vaccination that may damage cerebrospinal white matter. Rarely, it's accompanied by cerebellar ataxia. Other signs and symptoms include headache, fever, vomiting, an altered LOC, paralysis, seizures, oculomotor palsy, and pupillary changes.

Friedreich's ataxia.Friedreich's ataxia affects the spinal cord and cerebellum and causes gait ataxia, followed by truncal, limb, and speech ataxia. Other signs and symptoms include pes cavus, kyphoscoliosis, cranial nerve palsy, and motor and sensory deficits. A positive Babinski's reflex may appear.

Guillain-Barré syndrome.Peripheral nerve involvement usually follows a mild viral infection, rarely leading to sensory ataxia. Guillain-Barré syndrome also causes ascending paralysis and possibly respiratory distress.

Hepatocerebral degeneration.Patients who survive hepatic coma are occasionally left with residual neurologic defects, including mild cerebellar ataxia with a wide-based, unsteady gait. Ataxia may be accompanied by an altered LOC, dysarthria, rhythmic arm tremors, and choreoathetosis of the face, neck, and shoulders.

Multiple sclerosis (MS).Nystagmus and cerebellar ataxia commonly occur in MS, but they aren't always accompanied by limb weakness and spasticity. Speech ataxia (especially scanning) may occur as well as sensory ataxia from spinal cord involvement. During remissions, ataxia may subside or may even disappear. During exacerbations, it may reappear, worsen, or even become permanent. MS also causes optic neuritis, optic atrophy, numbness and weakness, diplopia, dizziness, and bladder dysfunction.

Olivopontocerebellar atrophy.Olivopontocerebellar atrophy produces gait ataxia and, later, limb and speech ataxia. Rarely, it produces an intention tremor. It's accompanied by choreiform movements, dysphagia, and loss of sphincter tone.

Poisoning.Chronic arsenic poisoning may cause sensory ataxia, along with headache, seizures, an altered LOC, motor deficits, and muscle aching. Chronic mercury poisoning causes gait and limb ataxia, principally of the arms. It also causes tremors of the extremities, tongue, and lips; mental confusion; mood changes; and dysarthria.

Polyneuropathy.Carcinomatous and myelomatous polyneuropathy may occur before detection of the primary tumor in cancer, multiple myeloma, or Hodgkin's disease. Signs and symptoms include ataxia, severe motor weakness, muscle atrophy, and sensory loss in the limbs. Pain and skin changes may also occur.

Porphyria.Porphyria affects the sensory and, more frequently, the motor nerves, possibly leading to ataxia. It also causes abdominal pain, mental disturbances, vomiting, headache, focal neurologic defects, an altered LOC, generalized seizures, and skin lesions.

Posterior fossa tumor.Gait, truncal, or limb ataxia is an early sign and may worsen as the tumor enlarges. It's accompanied by vomiting, headache, papilledema, vertigo, oculomotor palsy, a decreased LOC, and motor and sensory impairments on the same side as the lesion.

Spinocerebellar ataxia.With spinocerebellar ataxia, the patient may initially experience fatigue, followed by stiff-legged gait ataxia. Eventually, limb ataxia, dysarthria, static tremor, nystagmus, cramps, paresthesia, and sensory deficits occur.

Stroke.In stroke, occlusions in the vertebrobasilar arteries halt blood flow to cause infarction in the medulla, pons, or cerebellum that may lead to ataxia. Ataxia may occur at the onset of stroke and remain as a residual deficit. Worsening ataxia during the acute phase may indicate extension of the stroke or severe swelling. Ataxia may be accompanied by unilateral or bilateral motor weakness, a possible altered LOC, sensory loss, vertigo, nausea, vomiting, oculomotor palsy, and dysphagia.

Wernicke's disease.Wernicke's disease produces gait ataxia and, rarely, intention tremor or speech ataxia. With severe ataxia, the patient may be unable to stand or walk. Ataxia decreases with thiamine therapy. Associated signs and symptoms include nystagmus, diplopia, ocular palsies, confusion, tachycardia, exertional dyspnea, and orthostatic hypotension.

Other causes

Drugs.Toxic levels of anticonvulsants, especially phenytoin, may result in gait ataxia. Toxic levels of anticholinergics and tricyclic antidepressants may also result in ataxia. Aminoglutethimide causes ataxia in about 10% of patients; this effect usually disappears 4 to 6 weeks after drug therapy is discontinued.

» READ BOOK EXCERPT ONLINE »

Source: Nursing: Interpreting Signs and Symptoms, 2007

 » Next page: Symptoms of Spinocerebellar Ataxia

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