Sunday, May 7, 2017

Familial trembling of the chin--more than a half century ago

Wadlington WB. Familial trembling of the chin. J Pediatr. 1958. Sep;53(3):316-21.

Familial trembling of the chin has been described in four families in the literature. Grossman was the first to report this entity in the American literature. He traced the genealogy of his cases for four generations. Seven members exhibited quivering chins and no other detectable neurological abnormalities. Three members of the family also had otosclerosis. Various published pedigrees of families with otosclerosis  show that this gene is passed as a simple dominant, as is the gene which causes trembling chins...

In 1930 Frey  described five generations of a family with the abnormality of quivering chins. Their pedigree reveals there were 55 affected, 45 normal. Some of the children described by Frey had trembling of their chins noted in the first day of life. In others it did not start until 1 or 2 weeks of age and in others much later in life. An occasional member of the family reported would have an apparently spontaneous remission of his "disease" (as did the child [IV 2] reported in this communication). Still another female member of this family had experienced no abnormal movements of her chin for one year following termination of her first pregnancy when uncontrollable movements of her chin recurred especially during periods of anger or deep concentration. The only other abnormality in this family tree was one ease of nystagmus and several instances of intolerance for alcoholic beverages even in moderation…

The trembling of the chin may last for a few seconds or for several minutes. The tremor may be either tlne or coarse movements and not particularly related to the precipitating cause. The trembling of the chin is perpendicular and at the rate of two or three times per second. In most eases some emotional stimulus such as anxiety, anger, or depression acts as the trigger mechanism. It has been noted that the only stimuli which will act as a precipitating cause in some individuals are periods of concentration, awakening abruptly, or playing a violin…

Treatment for this condition has been unsatisfactory in the past. Frey used faradic current and ultraviolet light to no avail. Psychotherapy has been thought to be helpful at times. Since two of the children (IV 2, 4) had abnormal electroencephalograms, a therapeutic trial was made with anticonvu]sant therapy. ..

Since anxiety and emotional upsets have acted as trigger mechanism for many of these attacks in the past, it was decided to discontinue the dilantin so that the effects of a tranquilizer could be evaluated. Accordingly, the above patients were started on hydroxyzine hydrocbloride (Atarax ~)…

SUMMARY

1. This is the second family to be reported in the American literature with familial trembling of the chin. 
2. This is the first report of electroencephalograms of these patients.
3. Further therapeutic trials with tranquilizers or anticonvulsant therapy should be evaluated.

See also:  Grossman BJ. Trembling of the chin; an inheritable dominant character.
Pediatrics. 1957 Mar;19(3):453-5.

Frey E.Ein streng dominant erbliches Kinnmuskelzitern  Deutsche Ztschr. Nervenh., 115 (1930), p. 9

Courtesy of a colleague


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