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SUMMARY The authors retrospectively identified 17 children with Down syndrome who developed infantile spasms, and analysed their etiology, EEG findings, response to medication, development and prognosis. Compared with symptomatic infantile spasms in the general population, which have a poor prognosis, these patients had a relatively benign outcome with regard to seizure control: only three of 16 survivors currently have seizures and seven of the 16 currently receive anticonvulsants. Developmental outcome was poorest in those with a superimposed hypoxic insult and in those who regressed developmentally at the onset of the spasms and did not regain developmental milestones. The overall neurological prognosis for children with Down syndrome and infantile spasms appears to be better than for children with infantile spasms in the general population. RÉSUMÉ Spasmes en flexions chez les trisomiques 21 Les auteurs ont identifyé rétrospectivement 17 trisomiques 21 ayant présenté des spasmes en flexion et ils ont analyséľétiologie, les données EEG, les réponses aux médications, le développement et le pronostic. Comparés avee les spasmes infantiles symptomatiques de la population générale, que ont un pronostic sombre, ces patients présentatient un devenir relativement favorable en ce qui concernait le contrôle des crises: seulement trois des 16 survivants présentaient des crises et sept parmi ces seize recevaient une médication anticomitiale. Le devenir développemental était au plus bas en cas de lésion hypoxique surajoutée, ou en cas de régression au début des spasmes, sans récupération des étapes de développement par la suite. Le pronostic neurologique global pour les trisomiques 21 avec spasmes infantiles semble meilleur que chez les enfants à spasmes infantiles de la population générale. ZUSAMMENFASSUNG BNS Krämpfe bet Kindern mil Down Syndrom Die Autoren haben retros'pektiv 17 Kinder mit Down Syndrom untersuch't, die BNS Krämpfe entwickelten, und haben Aetiologie, EEG‐Befunde, Reaktionen auf Medikamente, Entwicklung und Prognose analysiert. Verglichen mit den symptomatischen BNS Krämpfen in der Allgemein‐bevölkerung, die eine schlecthe Prognose haben, hatten diese Patienten ein relativ gutes Ergebnis bei der Krampfkontrolle: nur drei der 16 Überlebenden haben laufend Anfälle und sieben der 16 antikonvulsiv behandelt. Der Outcome der Entwicklung war bei den Kindern mit einem über‐lagernden hypoxischen Insult und bei denen, die zu Beginn der Krämpfe Entwicklungsrückschritte machten und KEINE Entwtcklunsmeilenensteine mehr erreichten, am schlechtesten. Die allgemeine neurologische Prognose fur Kinder mit Down Syndrom und BNS Krämpfen scheint besser zu sein als für Kinder der Allgeneinbevölkerung mit BNS Krämpfen. RESUMES Espasmos infantiles en nifios con sindrome de Down Los autorcs identificaron retrospectivamente 17 nifios con sindrome de Down que sufrian espasmos infantiles y analizaron su etiologia, EEG, la respuesta a la medicacion, el desarrollo y el pronóstico. En comparacion con los espasmos infantiles sintomatieo...

A 6-year-old boy with a family history of hemiplegic migraine had a hemiplegic migraine lasting for 6 days complicated by prolonged fever, lethargy, and two brief focal seizures. An acute single photon emission computerized tomogram (SPECT) demonstrated decreased blood flow in the symptomatic cerebral hemisphere as well as crossed cerebellar diaschisis not previously documented in migraine. Another unique finding was the MRI with enhancement of the meninges and pial vessels over the symptomatic cerebral hemisphere. These findings suggest cerebellar and extra-axial involvement as components of hemiplegic migraine.Key words: hemiplegic migraine, cerebellar diaschisis, meningeal enhancement Abbreviations:FHM familial hemiplegic migraine, SPECT single photon emission computerized tomography, CCD crossed cerebellar diaschisis (Headache 1997;37:590-593) Familial hemiplegic migraine (FHM) is an autosomal dominant syndrome mapped to a gene encoding a brain-specific calcium ion channel on chromosome 19. 1 Its clinical phenotype can be quite variable. Mildly affected family members suffer attacks which can consist of hemiparesis, hemiparesthesia, and hemianopic blurring followed by a severe pulsatile headache. Other family members may suffer from severe attacks with drowsiness, confusion, coma, and hyperpyrexia accompanying the hemiparesis which may last for several days. We report a child with FHM whose prolonged attack allowed extensive evaluation by CT, MRI, EEG, and single photon emission computerized tomography (SPECT). CASE HISTORYThis previously vigorous 6-year-old boy fell while at play without immediate sequelae. One hour later, he complained of left hand numbness and headache. Throughout the night, he had several episodes of emesis without rigors, chills, or stiff neck.Remote past history revealed that he had a pyIoric stenosis and type 1 truncus arteriosus repair in infancy. Eight months prior to his current illness, he had an hour-long episode of altered consciousness after bumping his head on the playground. Details of the episode were vague because the event occurred at school, and the child was nearly back to baseline by the time the mother arrived. Neurologic examination, EEG, and CT of the brain I month later were normal and no specific diagnosis was made.The family history revealed that his mother had frequent episodes of left-sided numbness and hand clumsiness followed by severe headaches from age 5 to age 23. Her father had a history of a prolonged coma after a concussion while playing football as a teenager. There was no seizure history.Physical examination revealed a well-nourished pale child with a temperature of 38.9°C who lay on the bed with eyes closed. His cardiac examination revealed a harsh 3/6 holosystolic murmur which radiated to the back. Abdominal and lung examinations were unremarkable. Mental status was significant for somnolence and irritability. He could state his name and name toys as well as follow simple commands. Cranial nerve examination revealed a left homonymous hemianopsia a...

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