Patients with ataxia due to cerebellar atrophy often have the following:

Symptoms of Ataxia

Gait/Posture abnormalities - Difficulty maintaining normal upright posture, balance, coordinated walking, and running. Unsteady gait, staggering, tripping, falling, unsteadiness on stairs or maintaining balance on moving platforms, such as escalators or boats. These difficulties are often due to cerebellar dysfunction.

Fine motor incoordination - Difficulty with handwriting, cutting food, opening jars, buttoning clothes, sewing, typing, playing an instrument or a sport.

Speech and swallowing difficulties - Speech: slurred, slow, indistinct, abnormal in rhythm. Swallowing: difficulty swallowing or choking (especially with liquids).

Visual abnormalities - Blurred vision or double vision. Reading: difficulty moving from word to word. Problems following moving objects or shifting gaze from one object to another.

Increased fatigue - Patients with ataxia due to cerebellar atrophy often experience unexpected fatigue when performing normal activities. The impaired regulation of coordinated movements may lead to increased fatigue because of the need to expend more effort to perform activities that are no longer fluid or coordinated. Patients with ataxia often report needing to "concentrate on" their movements.

Cognitive and Mood Problems - In addition to motor dysfunction, patients with cerebellar degeneration may have cognitive and emotional difficulties. The cerebellum plays a role in some forms of thinking. Patients with cerebellar atrophy may have impaired recall of newly learned information or difficulty with "executive functions" such as making plans and keeping thoughts in proper sequence. Personality and mood disorders, such as increased irritability, anxiety, and depression, are more common in persons with cerebellar degeneration than in control subjects. It is important to examine mood and cognitive function in patients with cerebellar problems. These disorders are likely to respond to treatment, such as cognitive exercises, psychotherapy, and pharmacological treatment.

Patients with cerebellar ataxia may have symptoms that involve many aspects of life and vary significantly from person to person. Patients with ataxia should remain in close contact with a primary physician, neurologist, psychiatrist or psychologist, and with physical, speech and swallowing, and occupational therapists so that new difficulties can be recognized and treated early to reduce future injury.

 

It is important to examine mood and cognitive function in patients with cerebellar problems.


Persistent cerebellar ataxia with cerebellar cognitive

affective syndrome due to acute phenytoin

intoxication: A case report

Meena Gupta DM, Yogesh Patidar DM, Geeta A. Khwaja

DM, Debashish Chowdhury DM,*Amit Batra DM, Abhijit Dasgupta MD

Department of Neurology, GB Pant Hospital, New Delhi; *Max Super Speciality Hospital, New Delhi, India

 

Abstract

Phenytoin is one of the commonly used antiepileptic drugs. The common dose dependent and reversible neurological side effects of phenytoin are nystagmus, diplopia, dysarthria, ataxia, incoordination, chorioathetosis, orofacial dyskinesias and drowsiness. Persistent cerebellar dysfunction with cerebellar atrophy is a well known complication of long term phenytoin use.

 

There are several mechanisms proposed including hypoxia due to frequent seizures or toxic effects of phenytoin on cerebellar Purkinje cells. However, irreversible cerebellar dysfunction following acute phenytoin intoxication is rare.

 

We report a 20 year old female who presented with nystagmus, dysarthria, limb and truncal ataxia with orofacial dyskinesias and chorea. She also had cognitive and affective symptoms in the form of reduced attention, slow responses, lalling speech, blunting of affect, inappropriate laughter, reduced self care and executive dysfunction.

 

The symptoms started 2 weeks following the initiation of phenytoin 300mg/day, given prophylactically following left basal ganglia bleed. Her serum phenytoin was in toxic range, hence phenytoin was stopped. Her PET scan revealed bilateral cerebellar hypometabolism. At 6 months follow up, she had persistent ataxia with cognitive and affective dysfunction and follow up MRI showed diffuse cerebellar atrophy.

The clinical and radiological findings suggest that acute phenytoin intoxication is responsible for persistent ataxia and cerebellar cognitive affective syndrome.