Ataxia
Ataxia: Excerpt from Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series
Ataxia is defined as an incoordination and irregularity of voluntary, purposeful movements. Ataxia can be classified as cerebellar or sensory. Cerebellar ataxia is caused by disease of the cerebellum and its pathways between the cerebral cortex, the brain stem, and the spinal cord. Gait, trunk, limb and, possibly, speech abnormalities may occur in cerebellar ataxia. In sensory ataxia, position sense (proprioception) is impaired due to interruption of afferent nerve fibers in the peripheral nerves, posterior roots, posterior columns of the spinal cord, or medial lemnisci. Although uncommon, sensory ataxia may also be caused by a lesion in both parietal lobes. Sensory ataxia results in gait abnormalities. (See Identifying ataxia, page 34.)
Ataxia can be acute or chronic. Acute ataxia occurs with stroke, hemorrhage, or a large tumor in the posterior fossa. A large tumor in the posterior fossa is a life-threatening condition in which the cerebellum may herniate downward through the foramen magnum behind the cervical spinal cord, or upward through the tentorium of the cerebral hemispheres. Herniation may lead to compression of the brain stem. Acute ataxia may also result from drug toxicity or poisoning. Chronic ataxia can be progressive and may be seen with acute disease such as metabolic imbalances and with chronic degenerative neurologic disease.
Act Now: If ataxic movements develop abruptly, assess the patient for signs of increased intracranial pressure and impending herniation. Elevate the head of the bed. Determine the patient’s level of consciousness (LOC) and stay alert for pupillary changes, motor weakness or paralysis, neck stiffness, pain, and vomiting. Check the patient’s vital signs, noting rate and pattern of respirations. Have emergency resuscitation equipment readily available. Prepare the patient for a computed tomography scan or surgery.
Assessment
History
If the patient’s condition permits, obtain his history. Ask about the onset and initial presentation of ataxia, noting whether it developed suddenly or gradually. Note a history of multiple sclerosis, diabetes, central nervous system infection, neoplastic disease, previous stroke, or a family history of ataxia. Inquire about chronic alcohol abuse or prolonged exposure to industrial toxins such as mercury.
Physical examination
If the patient is able to stand, 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, and then closed. Minimal swaying indicates normal posture and balance, swaying and an inability to maintain balance with eyes open or closed suggests cerebellar ataxia, and increased swaying and an inability to maintain balance with eyes closed indicates sensory ataxia. Stand close to the patient during this test to provide support if he falls.
Evaluate motor strength when testing for gait and limb ataxia because motor weakness may mimic ataxic movements. Gait ataxia may be severe even when limb ataxia is minimal. Ask the patient with gait ataxia whether he tends to fall to one side and whether falls typically occur at night. With truncal ataxia, remember that the patient who has no symptoms while lying down but can’t walk or stand may appear to be experiencing hysteria or drug or alcohol intoxication.
Pediatric pointers
Ataxia occurs in acute and chronic forms in children. It can result from congenital or acquired disease. Acute ataxia may arise from febrile infection, brain tumors, mumps, and other disorders. Chronic ataxia may occur due to 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 his emotional state. Your examination may be limited to observing the child in spontaneous activity and carefully questioning his parents about such changes in motor activity as increased unsteadiness or falling. If you suspect ataxia, refer the child for a neurologic evaluation to rule out a brain tumor.
Geriatric pointers
Pre-existing diseases, such as dementia (Alzheimer’s type, vascular, or others) or previous stroke, may hamper an ataxia assessment. Functional problems may also impede the assessment of new symptoms.
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.
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.
Nursing considerations
Assess the patient’s neurologic status frequently. 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; refer him to psychiatric consultation if ordered. Promote rehabilitation goals set forth by the physical, occupational, and speech therapists, and implement safety measures. Refer the patient with progressive disease for counseling, if appropriate.
Patient teaching
Instruct the patient with sensory ataxia to move slowly, especially when turning or rising from a chair. Provide a cane or walker for extra support. Ask the patient’s family to assess his home for safety hazards, such as uneven surfaces or the absence of handrails on stairs. Refer the patient for home care follow-up nursing and rehabilitative services as ordered.
Pictures
Book Source Details
- Book Title: Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series
- Author(s): Springhouse
- Year of Publication: 2007
- Copyright Details: Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series, Copyright © 2007 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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