Ataxia

Ataxia: Excerpt from Professional Guide to Signs & Symptoms (Fifth Edition)

Classified as cerebellar or sensory, ataxia refers to incoordination and irregularity of voluntary, purposeful movements. Cerebellarataxia results from disease of the cerebellum and its pathways to and from the cerebral cortex, brain stem, and spinal cord. It causes gait, trunk, limb, and possibly speech disorders. Sensory ataxia results from impaired position sense (proprioception) due to interruption of afferent nerve fibers in the peripheral nerves, posterior roots, posterior columns of the spinal cord, or medial lemnisci or, occasionally, from a lesion in both parietal lobes. It causes gait disorders. (See Identifying ataxia.)

Ataxia occurs in acute and chronic forms. Acute ataxia may result from stroke, hemorrhage, or a large tumor in the posterior fossa. In this life-threatening condition, the cerebellum may herniate downward through the foramen magnum behind the cervical spinal cord, or upward through the tentorium on the cerebral hemispheres. Herniation may also compress the brain stem. Acute ataxia may also result from drug toxicity or poisoning. Chronicataxia can be progressive and may result from acute disease. It can also occur in metabolic and chronic degenerative neurologic disease.

Emergency interventions

History and physical examination

If the patient isn’t in distress, review his history. Ask about multiple sclerosis, diabetes, central nervous system infection, neoplastic disease, previous stroke, and a family history of ataxia. Also ask about chronic alcohol abuse or prolonged exposure to industrial toxins such as mercury. Find out if the ataxia developed suddenly or gradually.

If necessary, perform Romberg’s test to help distinguish between cerebellar and sensory ataxia. Instruct the patient to stand with his feet together and his arms at his side. Note his posture and balance, first with his eyes open and then with them closed. Test results may indicate normal posture and balance (minimal swaying), cerebellar ataxia (swaying and inability to maintain balance with eyes open or closed), or sensory ataxia (increased swaying and inability to maintain balance with eyes closed). Stand close to the patient during this test to prevent his falling.

If you test for gait and limb ataxia, be aware that motor weakness may mimic ataxic movements, so check motor strength, too. Gait ataxia may be severe, even when limb ataxia is minimal. Ask the patient with gait ataxia if he tends to fall to one side and if he falls more at night. With truncal ataxia, remember that the patient’s inability to walk or stand, combined with the absence of other signs while he’s lying down, may give the impression of hysteria or drug or alcohol intoxication.

Medical causes

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.

Special considerations

Prepare the patient for laboratory studies, such as blood tests for toxic drug levels and radiologic tests. Then focus on helping the patient adapt to his condition. Promote rehabilitation goals and help ensure the patient’s safety. For example, instruct the patient with sensory ataxia to move slowly, especially when turning or getting up from a chair. Provide a cane or walker for extra support. Ask the patient’s family to check his home for hazards, such as uneven surfaces or the absence of handrails on stairs. If appropriate, refer the patient with progressive disease for counseling.

Pediatric pointers

In children, ataxia occurs in acute and chronic forms and results from congenital or acquired disease. Acute ataxia may stem from febrile infection, brain tumors, mumps, and other disorders. Chronic ataxia may stem from Gaucher’s disease, Refsum’s disease, and other inborn errors of metabolism.

When assessing a child for ataxia, consider his level of motor skills and emotional state. Your examination may be limited to observing the child in spontaneous activity and carefully questioning his parents about changes in his motor activity, such as increased unsteadiness or falling. If you suspect ataxia, refer the child for a neurologic evaluation to rule out a brain tumor.

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Book Source Details

• Book Title: Professional Guide to Signs & Symptoms (Fifth Edition)
• Author(s): Springhouse
• Year of Publication: 2006
• Copyright Details: Professional Guide to Signs & Symptoms (Fifth Edition), Copyright © 2006 Lippincott Williams & Wilkins.

More About Cerebellar ataxia syndrome

• Back to disease: Cerebellar ataxia syndrome: Introduction
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More About This Book: Title: Professional Guide to Signs & Symptoms (Fifth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2006 ISBN: 1-58255-510-9

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