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When sleep-related hypermotor epilepsy (SHE) met Charles Darwin and Francis Galton.

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.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title neurology
Publication Year Start




PMID- 28760912
OWN - NLM
STAT- MEDLINE
DA  - 20170801
DCOM- 20170804
LR  - 20170804
IS  - 1526-632X (Electronic)
IS  - 0028-3878 (Linking)
VI  - 89
IP  - 5
DP  - 2017 Aug 01
TI  - When sleep-related hypermotor epilepsy (SHE) met Charles Darwin and Francis
      Galton.
PG  - 502-505
LID - 10.1212/WNL.0000000000004134 [doi]
AB  - 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.
CI  - (c) 2017 American Academy of Neurology.
FAU - Parrino, Liborio
AU  - Parrino L
AD  - From the Sleep Disorders Center (L.P.) and Unit of Neurology (G.P.), Azienda
      Ospedaliero-Universitaria di Parma, Italy. [email protected]
FAU - Pavesi, Giovanni
AU  - Pavesi G
AD  - From the Sleep Disorders Center (L.P.) and Unit of Neurology (G.P.), Azienda
      Ospedaliero-Universitaria di Parma, Italy.
LA  - eng
PT  - Biography
PT  - Historical Article
PT  - Journal Article
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
SB  - AIM
SB  - IM
MH  - Books/history
MH  - Epilepsy, Partial, Motor/genetics/*history
MH  - Genetic Predisposition to Disease
MH  - History, 19th Century
MH  - History, 20th Century
MH  - Humans
MH  - Sleep Wake Disorders/genetics/*history
MH  - United Kingdom
PS  - Darwin C
FPS - Darwin, Charles
PS  - Galton F
FPS - Galton, Francis
EDAT- 2017/08/02 06:00
MHDA- 2017/08/05 06:00
CRDT- 2017/08/02 06:00
PHST- 2016/11/18 [received]
PHST- 2017/04/13 [accepted]
AID - WNL.0000000000004134 [pii]
AID - 10.1212/WNL.0000000000004134 [doi]
PST - ppublish
SO  - Neurology. 2017 Aug 1;89(5):502-505. doi: 10.1212/WNL.0000000000004134.