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Catastrophic deterioration and hippocampal atrophy after childhood status epilepticus.

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Department of Neurology, Dokkyo University School of
Medicine, Tochigi, Japan
This research was supported in part by grants-in-aid from [he Ono
Medical Research Foundation, the Uehara Memorial Foundation,
the Ciba-Geigy Foundation (Japan) for the promotion of Science,
the Nakabayashi Trust for ALS Research, the Ryoichi Naito Foundation for Medical Research, and a Research Grant for Neuroimmunological Diseases from the Ministry of Health and Welfare of
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Catastrophic Deterioration and Hippocampal
Atrophy After Childhood Status Epilepticus
covery from the first episode was complete; however, after
the second, he had a catastrophic deterioration in language,
cognitive, and social skills. At age 9 years, formal neuropsychologic testing was impossible, owing to extremely poor expressive language, inattention, and autistic behavior.
A first cousin of the patient’s father has a history of bitemporal seizures. She also has psychiatric and cognitive dysfunction resembling the Kliiver-Bucy syndrome after a left
hemisphere stroke and left temporal lobe resecti~n.’,~The
father has another relative with epilepsy occurring after a hypoxic event and a relative with mental retardation. O n the
maternal side, one cousin has epilepsy and another has mental retardation. There is no consanguinity.
MRI volumetric studies showed marked hippocampal atrophy bilaterally. Hippocampal volumes, corrected for head
size, were 2,983.3 and 3,086.6 mm3, respectively, for the left
and right hippocampus (-6.3 SD below mean for the left
and -5.2 SD for the right). There were no other MRI abnormalities.
Our case supports DeLong and Heinz’s report of bilateral
hippocampal atrophy following prolonged status epilepticus
in childhood and the central role of the hippocampus in language and behavioral development. The unusual family history, particularly the illness of the father’s cousin, raises the
possibility that a genetic predisposition, possibly polygenic,
played a role in this patient’s disease. Disagreement exists
regarding whether seizures cause cerebral damage; however,
the close temporal relationship between the cognitive syndrome and status, along with the finding of hippocampal
atrophy, strongly suggest that, in these cases, status epilepticus caused the cognitive deterioration, as well as the hippocampal atrophy.*z5
Montreal Neurologic Institute and Hospital, Montreal,
Quebec, Canada
D. W. Gross, MD, FRCP(C), L. M. Li, MD,
and Frederick Andermann, MD, FRCP(C)
Delong and Heinz reported a series of 4 children who developed severe cognitive delay and bilateral hippocampal atrophy after prolonged status epilepticus in childhood. All
had been normal before status epilepticus. All were left with
severe language and cognitive delay after the episodes. Each
case had severe bilateral hippocampal atrophy o n magnetic
resonance imaging (MRI). W e document the following case
to lend support to these authors’ findings.
A boy, at ages 30 and 34 months, had episodes of status
epilepticus with mild fever. In both instances the seizures
were prolonged (the first episode lasted 90 minutes, whereas
the second required general anesthetic). Prolonged right
hemipareisis occurred after each episode. After the second
bout, he developed a habitual seizure disorder of temporal
origin with complex partial and generalized convulsions.
The child had been developmentally normal initially. Re-
Dr Gross acknowledges the financial support of the R. Samuel
McLaughlin Foundation.
1. DeLong GR, Heinz ER. The clinical syndrome of early-life bilateral hippocampal sclerosis. Ann Neurol 1997;42:11-17
2. Kluver H , Bucy PC. Preliminary analysis of functions of the
temporal lobes in monkeys. Arch Neurol Psych 1939;42:9791000
3. Scoville WB, Milner B. Loss of recent memory after bilateral
hippocampal lesions. J Neurol Neurosurg Psychiatry 1957;20:
4.Wasterlain CG. Recurrent seizures in the developing brain are
harmful. Epilepsia 1997;38:728-734
5. Camfield PR. Recurrent seizures in the developing brain are not
harmful. Epilepsia 1997;38:735-737
Annals of Neurology
Vol 43
No 5
May 1998
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statue, atrophy, epileptic, hippocampus, catastrophic, deterioration, childhood
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