Ataxia

Ataxia: Excerpt from Field Guide to Bedside Diagnosis

Differential Overview

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

Diagnostic Approach

Hemiparetic gait, a stroke residua, results from abduction/circumduction of the leg with a contralateral tilt of the body. Paraparetic gait, found with spinal cord disease or cerebral palsy, is marked by “scissoring,” or crossing with each step. A steppage gait, seen with peroneal neuropathy, has a foot drop with a high step to avoid toe dragging, and the foot slaps down. With a waddling gait, in proximal leg weakness such as myopathy, the leg is lifted high, and the trunk leans opposite. A Parkinsonian gait has a forward stoop with flexion of the hips, knees, and elbows and short shuffling steps, which accelerate. An apraxic gait, with bilateral frontal lobe disease, is shuffling, but the gait is hesitant and not maintained. A cerebellar/ataxic gait is broad-based and irregular. A sensory/ataxic gait is broadly based with a positive Romberg. With a vestibular gait, the patient falls to one side whether walking or standing. With hysterical gait, there is normal leg coordination while sitting but dramatic falls when standing.

A positive Romberg (unsteady with eyes closed, steady with eyes open) indicates posterior column disease. Gross lesions of the spinal cord rarely present with ataxia because of prominent weakness and spasticity. Cerebellar lesions produce dysmetria and decomposition of movement. Speech may be scanning, with each syllable pronounced separately.

With sensory ataxia, loss of touch causes little ataxia, but loss of proprioception causes severe ataxia, which increases with the eyes closed. The gait is wide-based with the feet landing with force. Both Romberg and pursuit (finger to nose or heel to shin) will be abnormal. It is caused by a lesion of the peripheral nerves, posterior columns (vitamin B₁₂ deficiency subacute combined degeneration), posterior roots (tabes), medial lemniscus, thalamus, or sensory cortex. Polyneuritis may be caused by diabetes, polyarteritis nodosa, alcohol, arsenic, Guillain-Barré, or porphyria.

Points of differentiation are as follows:

Clinical Findings

Vitamin B₁₂ deficiency  Combined system disease with involvement of posterior and lateral columns begins with weakness and paresthesia, which is followed by leg stiffness and ataxia. Loss of vibration and position sense is associated with upgoing toes and hyporeflexia in the legs. Associated mental status changes may occur.

Parkinsonism  Typical findings are a cyclic tremor, “cog-wheeling,” and increased tone. The patient moves slowly with a stooped posture, shuffling gait, decreased limb movement, and slightly flexed hips and knees, turning en bloc. The pace gradually increases (festination).

Myopathy  Proximal motor weakness is evident, with a waddling gait, in which the foot is lifted high and the trunk leans opposite with each step.

Cervical spondylosis  It is marked by neck and arm pain with upgoing toes.

Multiple sclerosis  It most commonly presents with optic neuritis or flaring and remitting patchy numbness separated in time and space. Eye findings include optic neuritis (acute with a hyperemic disc or old with a white atrophic disc), nystagmus, and internuclear ophthalmoplegia. Lhermitte sign, an electric sensation with neck flexion, may be present. Autonomic findings of urgency, incontinence, or impotence, and reflex findings of spasticity, weakness, or clonus may be found.

Multiple subcortical strokes  These are recognized by a stepwise pattern and the associated emotional lability, brisk reflexes, increased jaw jerk, and dysarthria.

Alcoholic cerebellar degeneration  It is characterized by ataxia of the legs with less prominent involvement of the arms, speech, or ocular motility. Polyneuropathy is usually present.

Hydrocephalus  Ataxia is combined with memory loss and incontinence.

Frontal lobe tumor  “Frontal ataxia” with a tendency to fall backward, signs of cerebellar disease, grasp and snout reflexes, incontinence, and slow mentation are features.

Cerebellar tumor  Cerebellar disease produces ataxia and decomposition of movement. Involvement of the lateral lobe elicits limb ataxia (especially upper) and hypotonia. Involvement of the flocculonodular lobe causes truncal ataxia, drunken gait, and titubation of the head and trunk. If the anterior lobe is affected, there will be gait ataxia, with inability to tandem walk. Lesions of the midline cerebellum or vermis make the patient unable to stand or walk due to ataxia and dysequilibrium, but use of limbs is normal and there is no nystagmus when sitting or lying. Tumors are usually metastatic in adults.

Spinocerebellar degeneration  A family history is prominent, and there are widespread deficits including hyporeflexia and upgoing toes. Pes cavus is often found.

Syringomyelia  Prominent features include sensory disassociation with loss of pain and temperature sensation but preservation of touch and position, anterior horn cell involvement with muscle wasting, fasciculations, absent reflexes in the upper extremities, and corticospinal tract involvement with spasticity.

Tabes dorsalis  The gait is a wide-based ataxia with foot slapping. Associated findings include loss of position, deep pain and temperature sensation, areflexia, and Argyll Robertson pupils, which accommodate but do not react to light.

Chorea  There is decreased tone with marked hyperextensibility of joints. The gait is wide-based and lurching with excessive abnormal movements and posturing.

Alcohol intoxication  There is a wide-based cerebellar gait in a patient who appears inebriated and has an alcohol odor to the breath.

Labyrinthitis  Vertigo is a prominent feature, which is exacerbated by head position. The patient will fall to one side.

Cerebellar hemorrhage  It presents with sudden onset of occipital headache, vertigo, and ocular gaze palsies, with preserved limb strength and sensation.

Cerebellar infarct  The presentation is similar to cerebellar hemorrhage, but with prominent limb, trunk, gait, and speech ataxia, nystagmus, and hypotonia.

Guillain-Barré syndrome  Ataxia often develops in the early stages. Reflexes will be absent.

Hysterical  The ataxia is inconsistent, varying from moment to moment, requiring more coordination than usual.

Parietal apraxia  The patient “forgets” how to walk, but results of formal testing are normal.

Pictures

Book Source Details

• Book Title: Field Guide to Bedside Diagnosis
• Author(s): David S. Smith
• Year of Publication: 2007
• Copyright Details: Field Guide to Bedside Diagnosis, Copyright © 2007 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: Field Guide to Bedside Diagnosis Authors: David S. Smith Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 0-78178-165-5

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