TY - JOUR
T1 - The origin of hypsarrhythmia and tonic spasms in West syndrome
T2 - Evidence from a case of porencephaly and hydrocephalus with focal hypsarrhythmia
AU - Haginoya, Kazuhiroa
AU - Kon, Kimiya
AU - Tanaka, Soichiro
AU - Munakata, Mitsutoshi
AU - Kato, Rie
AU - Nagai, Mayumi
AU - Yokoyama, Hiroyuki
AU - Maruoka, Shin
AU - Yamazaki, Tetsuro
AU - Iinuma, Kazuie
N1 - Funding Information:
This study was supported in part by Grant-in-Aid from the Ministry of Education, Science and Culture, and from the National Center of Neurology and Psychiatry (NCNP) of the Ministry of Health and Welfare, Japan.
PY - 1999/3/1
Y1 - 1999/3/1
N2 - 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. Copyright (C) 1999 Elsevier Science Ireland Ltd.
AB - 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. Copyright (C) 1999 Elsevier Science Ireland Ltd.
KW - Hypsarrhythmia
KW - Infantile spasms
KW - Single photon emission computed tomography
KW - Tonic spasms
KW - West syndrome
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U2 - 10.1016/S0387-7604(98)00082-5
DO - 10.1016/S0387-7604(98)00082-5
M3 - Article
C2 - 10206533
AN - SCOPUS:0032976773
VL - 21
SP - 129
EP - 131
JO - Brain and Development
JF - Brain and Development
SN - 0387-7604
IS - 2
ER -