A four year old boy with migraine associated with focal cerebral edema, CSF pleocytosis, and progressive cerebellar ataxia is reported from the Departments of Neurology and Radiology, Yale University School of Medicine, New Haven, CT. The onset was at one year of age with an episode of unresponsiveness which resolved within one day. At three years of age he suffered acute cortical blindness for 36 hours. At four years of age he presented with a pulsating left-sided headache and an acute right hemiparesis. An MRI showed increased signal in the left parietooccipital region, enhanced with Gadolinium. The next day he had a right focal seizure lasting five minutes and a seizure occurrence with persistent nonresponsiveness and right hemiparesis. On recovery over several days, the hemiparesis fluctuated, a left-sided headache resolved, but cerebellar ataxia developed and persisted. The CSF was clear with 60 WBC, protein 12 mg/dl, and glucose 75 mg/dl. A course of Propranolol has prevented recurrence of hemiparesis and headache but lower extremity spasticity and gait ataxia have persisted. [1]

COMMENT. A so-called meningitic migraine with cerebral edema and autosomal dominant cerebellar ataxia has been reported previously in an adult [2] but the present case is the first report in a child and with MRI documentation of a transient lesion. Severe prolonged migrainous symptoms and prolonged partial status epilepticus are characteristic features of the MELAS syndrome or mitochondrial encephalomyopathy which should be considered in the differential diagnosis of patients with headache and seizures. [3]

Dr. John Wilson and his colleagues at the Hospital for Sick Children, Great Ormond Street, London, have emphasized the importance of food intolerance and an allergic mechanism for migraine in children. As a visitor at Great Ormond Street for one year I was impressed with the results of hypoallergenic dietary treatment for migraine in children, and the benefits of this approach to treatment as a substitute for drug therapy have been confirmed in my pediatric neurology practice. The short term elimination of cow’s milk, egg, wheat cereals, chocolate, orange and cheese in the diet of the above child might be considered as a complement or substitute for the propranolol therapy.

Hockaday JM is the editory of a new book on “Migraine In Childhood” (Butterworth, Guildford, England 1989) which has received an excellent review by Dr. Richard O. Robinson. [4]