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Sleeping Beauty Syndrome
Sleeping Beauty Syndrome
Body:
The cause of Kleine-Levin syndrome is unknown, although the disorder
has a strong genetic component. This uncommon sleep disorder tends to run in
families, especially of Jewish ancestry. About 15 percent of the Kleine-Levin
syndrome patients are of Jewish origin and “the incidence reported in Israel is
unproportionately high,” according to a paper in a Jewish medical journal. “The
most promising findings are the familial clustering and a potential Jewish
founder effect, supporting a role for genetic susceptibility factors,” according
to French researchers. This disorder generally appears in adolescence and
subsides on its own. Over time, episodes diminish in frequency and severity,
becoming less disruptive to the individual and family. On average, it takes 8-14
years for patients to “outgrow” Kleine-Levin syndrome, with longer duration in
men, in patients with hypersexuality, and when onset is after age 20.
This condition, although rare, appears to affect adolescent males who are
typically in their teenage years (16 years old). In fact, out of everyone who has
this condition, around 70 percent are male. Women, however, tend to keep the
disorder longer men. Even though there are over 500 reported cases of this
disorder in medical literature, it still often goes under-diagnosed and
unrecognized, which makes it challenging to truly figure out its frequency in
the overall population. More than 500 cases have been reported in the medical
literature. However, because cases of Kleine-Levin syndrome often go
unrecognized, the disorder is under-diagnosed, making it difficult to determine
its true frequency in the general population. In some cases, symptoms subside
with advancing age (spontaneous remission), most often by early adult life.
However, in other cases, the disorder persists throughout adulthood.
Conclusion:
KLS is a neurological disorder with a lot of psychiatric coloring, therefore, a case
may present to a neurologist, physician, or psychiatrist depending on patients’
awareness, feasibility, and predominant symptoms. Hence, it is important for
clinicians to have high index of suspicion on such atypical presentations. Any
case presenting with episodic hypersomnolence should be evaluated thoroughly
with multidisciplinary approach for timely diagnosis and rational treatment.
Kleine Levin Syndrome (KLS) or Sleeping Beauty Syndrome patient experiences
recurrent episodes of excessive sleep, episodes occur at least once a year, and
are generally 2 days to 4 weeks in duration and during episodes, when awake,
cognition is abnormal with feeling of unreality or confusion. Behavioral
abnormalities such as megaphagia or hypersexuality may occur in some
episodes. The patient has normal alertness, cognitive functioning, and behavior
between the episodes, the condition is not better accounted for by another
mental disorder (e.g, mood disturbance), and is not due to the direct
physiological effects of a substance (e.g., a drug of abuse, a medication) or
another general medical condition (e.g. a metabolic disorder). There is increasing
evidence that the disorder is a genuine disease entity based on its consistent
description in term of clinical presentation, demographics (70% male,
adolescent), evolution (eventually disappears), and therapeutic response (almost
nothing is effective). It may be misdiagnosed as depression or other psychiatric
consequences, with devastating consequences.
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