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Arch Neurol. 2003;60:663-664.

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IN THIS ISSUE of the ARCHIVES, Kors et al¹ describe the clinical features of patients with familial hemiplegic migraine (FHM) in 5 unrelated families. They point out that a threonine-to-methionine substitution at position 666 in the polypeptide chain of the ₁-subunit of the neuronal calcium channel (T666M) is the most frequent mutation in autosomal dominant FHM. All neurologists know that migraine symptoms range from mild to severe and that a family history of similar headaches is very common. The reader would assume that a well-defined mutation should convey identical clinical manifestations. Kors and colleagues show that this assumption is incorrect. In addition to migraine and hemiplegia, their patients had 1 or more of the following: confusion, coma, progressive cognitive decline, aggressive behavior, aphasia, ataxia, anisocoria, fever, and spinal fluid pleocytosis. Imaging studies showed a frank, ultimately fatal cerebral infarction in one patient and cerebellar atrophy in another. Cerebral angiography caused . . . [Full Text of this Article]

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RELATED ARTICLE

Expanding the Phenotypic Spectrum of the CACNA1A Gene T666M Mutation: A Description of 5 Families With Familial Hemiplegic Migraine
E. E. Kors, J. Haan, N. J. Giffin, L. Pazdera, C. Schnittger, G. G. Lennox, G. M. Terwindt, F. L. M. J. Vermeulen, A. M. J. M. Van den Maagdenberg, R. R. Frants, and M. D. Ferrari
Arch Neurol. 2003;60(5):684-688.
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