Clinical History

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A positive family history suggests a form of inherited ataxia. Different ataxic disorders start in infancy, childhood, or adulthood. Patients and relatives should be asked about delayed motor milestones or poor athletic performance at school. The course of the disease might be of acute (over minutes or hours) or subacute (over days or weeks) onset, nonprogressive from birth, and episodic or slowly progressive (over month or years). By definition, congenital ataxias are nonprogressive since birth. [aJ The acute onset of severe headache in association with ataxia suggests a diagnosis of cerebellar hematoma or ischemia. A recent history of trauma, such as a fall on the back of the head, suggests hematoma or craniocerebral injury. Subacute onset is found in viral cerebellitis, multiple sclerosis, paraneoplastic cerebellar syndromes, posterior fossa tumors or abscesses, and alcoholic cerebellar degeneration. Episodic ataxia in adults might be caused by drug ingestions, multiple sclerosis, transient vertebrobasilar ischemic attacks, foramen magnum compression, intermittent obstruction of the ventricular system, and dominantly inherited periodic ataxia. In children and young adults, a metabolic disorder should be expected. Chronic progressive ataxia suggests an inherited or idiopathic degenerative ataxia.

Patients should be asked if symptoms are worse in the dark. Ataxia largely due to sensory loss or vestibular disease is typically enhanced by lack of visual feedback compared with cerebellar ataxia. Facial droop, vertigo, hearing loss, numbness, or diplopia associated with ataxia suggest a disorder in the brain stem. The history of previous subacute monocular visual loss suggests a diagnosis of multiple sclerosis. Some degenerative ataxias are associated with gradual visual loss. Occipital headache and projectile vomiting in conjunction with ataxia are associated with raised intracranial pressure in the presence of a posterior fossa lesion. Headache and vomiting are commonly worse on coughing, bending, and walking and in the morning. Incontinence associated with dementia and gait ataxia suggests normal pressure hydrocephalus. Postural hypotension and extrapyramidal signs are associated with multiple system atrophy. Skeletal abnormalities like scoliosis and pes cavus, as well as diabetes and cardiac symptoms, are associated with Friedreich's ataxia. Repeated infections are associated with ataxia telangiectasia.

Patients should be asked whether they are receiving antiepileptic (phenytoin, carbamazepine) or cytotoxic drugs (fluorouracil, cytarabine), lithium, or alcohol, and whether they have been exposed to any toxins (heavy metals, solvents). Ataxia might be associated with primary nervous system tumors such as astrocytomas and medulloblastomas and malignant disorders (bronchial and ovarian carcinoma, Hodgkin's disease), infections (varicella, Epstein-Barr virus), or hypothyroidism.


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