Diseases » Spinocerebellar Ataxia

Ataxia

Ataxia: Excerpt from Signs & Symptoms: A 2-in-1 Reference for Nurses

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, is caused by a lesion in both parietal lobes. It causes gait disorders. ( See Identifying ataxia, page 66.)

Ataxia occurs in acute and chronic forms. Acute ataxia may result from stroke, hemorrhage, or a large tumor in the posterior fossa. With 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, at times, can result from acute disease. It can also occur in metabolic and chronic degenerative neurologic disease.

Emergency Actions

If ataxic movements suddenly develop, examine the patient for signs of increased intracranial pressure and impending herniation. Determine his level of consciousness (LOC), and be alert for pupillary changes, motor weakness or paralysis, neck stiffness or pain, and vomiting. Check vital signs, especially respirations; abnormal respiratory patterns may quickly lead to respiratory arrest. Elevate the head of the bed. Have emergency resuscitation equipment readily available. Prepare the patient for computed tomography scanning or surgery.

History

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 patient’s ataxia developed suddenly or gradually.

Physical assessment

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 sides. Note his posture and balance, first with his eyes open, then 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 and check motor strength, too. Gait ataxia may be severe, even when limb ataxia is minimal. With gait ataxia, ask the patient if he tends to fall to one side and if falling usually occurs at night. With truncal ataxia, remember that inability to walk or stand, combined with the absence of other signs when 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

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.

Special considerations

Prepare the patient for laboratory studies such as blood tests for toxic drug levels and radiologic tests. Treatment approaches depend on the underlying cause. For example, physical therapy may improve function following a stroke. Surgery, chemotherapy, and radiation therapy may be necessary to treat a brain tumor. If toxic drug levels are the cause, discontinue the offending drug.

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.

Patient counseling

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.

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Book Source Details

• Book Title: Signs & Symptoms: A 2-in-1 Reference for Nurses
• Author(s): Springhouse
• Year of Publication: 2007
• Copyright Details: Signs & Symptoms: A 2-in-1 Reference for Nurses, Copyright © 2007 Lippincott Williams & Wilkins.

More About Spinocerebellar Ataxia

• Back to disease: Spinocerebellar Ataxia: Introduction
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

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More About This Book: Title: Signs & Symptoms: A 2-in-1 Reference for Nurses Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-58255-318-1

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