Choreoathetosis as a side effect of gabapentin therapy in severely neurologically impaired patients.

OBJECTIVE: To evaluate the relationship of gabapentin therapy with choreoathetotic movements in mentally retarded patients treated with intractable epilepsy. DESIGN: Case reports of 2 institutionalized patients who developed choreoathetosis temporally related to adjunctive therapy with gabapentin at dosages of 1200 to 1800 mg/d. RESULTS: Both patients experienced resolution of abnormal movements on discontinuation of the therapy. One patient developed recurrent choreiform movements after drug rechallenge. CONCLUSION: We suggest that, in patients with mental retardation and epilepsy, involuntary movements may either occur as reversible side effects of gabapentin therapy or result from a previously undescribed adverse drug interaction with other antiepileptic agents.

Gabapentin for familial paroxysmal dystonic choreoathetosis.

We present a 4-year-old girl with stereotyped episodes of inability to speak and dystonic posturing of the face and extremities lasting 20 minutes. An older brother and mother had similar spells in childhood. Routine and video-EEG during events were normal. The diagnosis was non-kinesigenic paroxysmal dystonic choreoathetosis since the episodes were not exacerbated by movement. Gabapentin 10 mg/kg/d eliminated most attacks.

Abnormal sudomotor function in the hypomelanotic macules of tuberous sclerosis complex.

To investigate the integrity of sympathetic innervation in the hypomelanotic macules of tuberous sclerosis complex, we studied sudomotor function in nine patients with tuberous sclerosis complex. Postganglionic sudomotor function was assessed using the Silastic imprint test in nine patients with tuberous sclerosis complex who have at least one hypomelanotic macule greater than 2 cm in diameter. Sweating was induced by iontophoresis with 0.5% pilocarpine nitrate and sweat droplets were counted under a microscope using a 1 x 1 cm grid. Silastic imprint testing of an analogous skin area contralateral to the hypomelanotic macule was measured as a control. Sweat volume quantitation using sweat collectors was performed in five of the subjects. The sweat volume collected from the hypomelanotic macule was reduced compared to the control skin in four of the five subjects. Sweat droplet counts from the hypomelanotic macule were significantly reduced in only one of nine subjects. These data suggest that, although there is no difference in the number of functioning sweat glands in most hypomelanotic macules, the sweat glands produce less sweat (ie, decreased sweat volume) than in normal skin. We hypothesize that focal abnormalities of sympathetic innervation might be responsible for the hypomelanotic macules of tuberous sclerosis complex.

Acute onset of chorea and dystonia following a febrile illness in a 1-year-old boy.

A 12-month-old boy with acute onset hemichorea and dystonia following a gastroenteritis has abnormal signal intensities of his basal ganglia on brain magnetic resonance imaging (MRI). A rigorous laboratory investigation is successful in diagnosing his rare condition. A discussion of the differential of abnormal basal ganglia on MRI is presented to help illustrate this case.