Neuroleptic malignant syndrome-like state in a patient with Down syndrome and basal ganglia calcification.
We report a case of rhabdomyolysis in a 13-year-old Down syndrome patient with progressive quadriplegia, choreoathetosis and dystonia. Cranial CT demonstrated bilateral basal ganglia calcification. He experienced the sudden onset of high fever, cloudiness of consciousness, muscle rigidity and severe opisthotonus. The diagnosis was made on the basis of the marked increases in serum creatine kinase and myoglobin. There was remarkable elevation of 5-hydroxyindoleacetic acid, homovanillic acid and methoxy-hydroxyphenyl glycol in the cerebrospinal fluid during hyperpyrexia. This case exhibited almost all the diagnostic criteria of the neuroleptic malignant syndrome. It was suggested that abnormalities of monoamines in the central nervous system may be related to the pathologic etiology of this state and rhabdomyolysis.
Komatsu
H
H
Department of Pediatrics, Kyoto Prefectural University of Medicine, Japan.
Nishimura
A
A
Okano
S
S
Miyanomae
Y
Y
Takeuchi
Y
Y
Sawada
T
T
D000293:Adolescent; D001480:Basal Ganglia Diseases / Q000150:complications*; D002114:Calcinosis / Q000150:complications*; D004314:Down Syndrome / Q000150:complications*; D006719:Homovanillic Acid / Q000134:cerebrospinal fluid; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000134:cerebrospinal fluid; D008297:Male; D008734:Methoxyhydroxyphenylglycol / Q000134:cerebrospinal fluid; D009459:Neuroleptic Malignant Syndrome / Q000175:diagnosis*; D012206:Rhabdomyolysis / Q000134:cerebrospinal fluid / Q000209:etiology* / Q000503:physiopathology
D008734:Methoxyhydroxyphenylglycol; D006897:Hydroxyindoleacetic Acid; D006719:Homovanillic Acid