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Westphal's syndrome
Also known as:
Cavaré-Romberg syndrome
Cavaré-Romberg-Westphal syndrome
Cavaré-Westphal syndrome
Westphal’s disease
Westphal’s neurosis
Associated persons:
C. Cavaré
Moritz Heinrich Romberg
Karl Friedrich Otto Westphal
Description:
A familial form of intermittent hypocalcaemic paralysis. It is characterized by periodic attacks of flaccid paralysis of the extremities and trunk, in association with complete areflexia and lack of electrical excitability of the muscles and. Onset usually between the ages of 7 and 21 years. The attacks can be precipitated by infusions of glucose and insulin, eating a large meal, or administration of epinephrine. A rare disorder transmitted as an autosomal dominant trait. Reduced penetrance and expressivity in women.
Previous reports of similar cases by William Musgrave (1657-1721), C. Cavaré 1853, Moritz Heinrich Romberg 1857 (in his Lehrbuch der Nervenkrankheiten [Textbook of nervous diseases] 3rd edition), and Hartwig 1874 (the only Hartwig I have found is the German obstetrician Karl Hartwig, 1844-1895).
Bibliography:
- W. Musgrave:
A periodic palsy.
The philosophical transactions and collections to the end of the year 1700. 2: 33.
- C. F. O. Westphal:
Über einen merkwürdigen Fall von periodischer Lähmung aller vier Extremitäten mit gleichzeitigem Erlöschen der elektrischen Erregbarkeit während der Lähmung.
Berliner Klinische Wochenschrift, 1885, 22: 489-491, 509-511.
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