When sleep-related hypermotor epilepsy (SHE) met Charles Darwin and Francis Galton.
Neurology
; 89(5): 502-505, 2017 Aug 01.
Article
in En
| MEDLINE
| ID: mdl-28760912
ABSTRACT
Sleep-related hypermotor epilepsy (SHE) is characterized by short-lasting seizures patterned by repetitive and stereotyped motor events in the same person. In autosomal dominant SHE, genetic factors play a well-known key role. In The Expression of Emotions in Man and Animals, Charles Darwin quotes a plausible example of SHE illustrated by his cousin Sir Francis Galton "the gentleman
lay fast asleep on his back in bed, raising his right arm slowly in front of his face, up to his forehead, and then dropping it with a jerk, so that the wrist fell heavily on the bridge of his nose. The trick did not occur every night, but occasionally, and was independent of any ascertained cause. Sometimes it was repeated incessantly for an hour or more." Similar manifestations during sleep occurred also in the patient's son and granddaughter, suggesting an autosomal inheritance without sex relationship. Differential diagnosis with REM behavior disorder and other parasomnias is discussed. To our knowledge, this could be the first description of a stereotyped SHE pattern with genetic transmission.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Sleep Wake Disorders
/
Epilepsy, Partial, Motor
Limits:
Humans
Country/Region as subject:
Europa
Language:
En
Journal:
Neurology
Year:
2017
Document type:
Article
Affiliation country:
Italy