The origin of hypsarrhythmia and tonic spasms in West syndrome: evidence from a case of porencephaly and hydrocephalus with focal hypsarrhythmia

We report on a 3-year-old girl with West syndrome and with focal hypsarrhythmia. The left hemisphere of the patient was virtually completely defective and continuous hypsarrhythmia was only seen in the residual right frontal cortex, where an interictal single photon emission computed tomography (SPE...

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Bibliographic Details
Published in:Brain & development (Tokyo. 1979) 1999-03, Vol.21 (2), p.129-131
Main Authors: Haginoya, Kazuhiroa, Kon, Kimiya, Tanaka, Soichiro, Munakata, Mitsutoshi, Kato, Rie, Nagai, Mayumi, Yokoyama, Hiroyuki, Maruoka, Shin, Yamazaki, Tetsuro, Iinuma, Kazuie
Format: Article
Language:English
Subjects:
Biological and medical sciences
Brain - abnormalities
Brain - diagnostic imaging
Brain - pathology
Brain - physiopathology
Brain Stem - blood supply
Brain Stem - diagnostic imaging
Cerebellum - blood supply
Cerebellum - diagnostic imaging
Electroencephalography
Female
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Humans
Hydrocephalus - complications
Hydrocephalus - diagnosis
Hypsarrhythmia
Infant, Newborn
Infantile spasms
Magnetic Resonance Imaging
Medical sciences
Nervous system (semeiology, syndromes)
Neurology
Regional Blood Flow - physiology
Single photon emission computed tomography
Spasm - complications
Spasm - diagnosis
Spasms, Infantile - complications
Spasms, Infantile - diagnosis
Tomography, Emission-Computed, Single-Photon
Tonic spasms
West syndrome
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Summary:We report on a 3-year-old girl with West syndrome and with focal hypsarrhythmia. The left hemisphere of the patient was virtually completely defective and continuous hypsarrhythmia was only seen in the residual right frontal cortex, where an interictal single photon emission computed tomography (SPECT) showed hyperperfusion. Despite a focal epileptic pattern, the tonic spasms were quite symmetrical. In our patient, spasms might not require the sensorimotor cortex, but the brainstem containing the descending pathways that control spinal reflexes and other infratentorial structures seem to be essential for the occurrence of spasms. This is in accordance with the result of an ictal SPECT that showed hyperperfusion of the brainstem and cerebellum. These findings suggest that hypsarrhythmia originates from cortical lesions, while subcortical structures may be primarily responsible for the tonic spasms in this patient.
ISSN:0387-7604
1872-7131
DOI:10.1016/S0387-7604(98)00082-5