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Headache characteristics in episodic ataxia

We report a 33-year-old English female, presented elsewhere with frequent headaches for few months. The headaches were incapacitating, involved either temporoparietal region, throbbing, lasting 4–5 h and associated with photophobia, phonophobia, and nausea and vomiting. There was no preceding aura or associated neurological deficits. She denied any past or family history of migraine. Headaches continued despite propranolol and ergot preparations. Few weeks later during an episode of headache, she complained of blurred vision and pain around left eye. Additionally, she had few falls that occurred without warning or loss of consciousness. Magnetic resonance imaging (MRI) of brain was normal. With a clinical diagnosis of retrobulbar neuritis, oral steroids were started. As there was no relief, she decided to come to our tertiary care center for further evaluation. At our center, she mostly preferred to stay in bed due to incapacitating headache, severe photophobia and a sense of unsteadiness. Initial neurological and ophthalmological examinations were unremarkable with no clinical evidence of retrobulbar neuritis. A mild weakness with Babinski’s response was noted in her left leg, which she related to horse riding injuries in childhood. The blood investigations, cerebrospinal fluid analysis and MRI studies of brain, cervical and thoraco-lumbar spine were unremarkable. No oligoclonal bands were seen in the cerebrospinal fluid. Various agents like amitryptiline, carbamazepine, propranolol and flunarazine in different combinations resulted in only partial and temporary relief. During one of the hospitalizations, some transient episodes of intermittent intention tremors of left hand, nystagmus, slurred speech and ataxia were observed. The episodic nature of ataxic attacks and unremarkable investigations led to a possible diagnosis of episodic ataxia and detailed family history was sought. She was found to be an adopted child with no details about her original parentage. She confessed of being clumsy child with frequent falls . Further genetic studies showed mutation in alpha-1 subunit of CACNA1Aconsistent with a diagnosis of Episodic Ataxia-2 (EA-2). Acetazolamide, 250 mg twice daily, resulted in a dramatic response in headache as well as ataxic attacks sustained over the subsequent 2 years of follow-up.