Patient’s when she would be forced to awaken. Her

Patient’s symptoms correlate with recurrent primary hypersomnia, highly
suspicious for Kleine-Levin syndrome (KLS). Other primary hypersomnias that may
be considered include narcolepsy.

Conditions that can mimic central hypersomnia such as myotonic dystrophy or
neurological disorders such as a brain tumor are unlikely and did not show up
on the MRI obtained at the outside hospital. Patient did not report cataplexy
suggestive of a narcolepsy syndrome and did not report excessive daytime
sleepiness in between episodes. There was no evidence for an insufficient sleep
syndrome or a history of a seizure disorder. Infectious encephalitis was
considered, but in the absence of other systemic symptoms and multiple episodes
over several years, this was highly unlikely.

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Symptoms of KLS may overlap with that of bipolar
depression, however this was unlikely as there were no hypomanic or manic
episodes in this patient. The irritability and occasional disinhibited
behaviors that can appear similar to hypomanic/manic or a mixed episodes
occurred exclusively in the hypersomnolence episodes and when she would be
forced to awaken. Her depression occurred weeks after her first episode and
worsened in the setting of hypersomnolence events. The depression during these events may be a consequence of
the actual Klein-Levin syndrome, and this may be a part of the stigmata of the
syndrome. The patient also developed anticipatory anxiety. These events significantly
disrupted her life, and she occasionally had partial recall of embarrassing disinhibited
behaviors.

 

Patient
was arousable, but became irritable when awakened. She was able to use the
restroom and eat when prompted. She experienced anterograde amnesia and dream-like
derealization. Patient had anorexia rather than hyperphagia which is more common
in KLS. Her mother described her as predominantly emotional during these
events if awakened. She also experienced visual hallucinations and
delusional-like thoughts during the episodes but not when she was in remission.

 

In
summary, this young female has symptoms highly suggestive of Kleine-Levin
syndrome. Based on the onset of her events, the characterization of the events
including the anorexia, irritability, reduced long-term working memory
following the episode.

General Remarks:

 

The
symptoms of Kleine-Levin syndrome (KLS) are characterized by their intermittent
and periodic nature. Because KLS is rare, other conditions with
similar symptoms are usually considered first and the condition is usually a
diagnosis of exclusion. In this case, patient’s mother was informed to bring
patient to the office if she has another episode for a thorough workup
including polysomnography, head MRI, urine drug screen and other serological
tests.

KLS usually occurs
as a 2:1 ratio in males. Episodes progress
fairly rapidly from time of onset and reach their symptomatic period within 24
hours. The episodes themselves usually last between 1 and 3 weeks. The classic triad of hyperphagia, hypersomnolence and
hypersexuality is not always present. In a case series, only 45% of KLS
patients had this triad. In our case, patient had anorexia and had no
reported symptoms of hypersexuality. Although
depression is not a core feature of KLS, some patients can become extremely
upset during episodes, and it is important to monitor mood.

Some studies support
the use of lithium as preventative therapy showing a decrease in both the
duration and intensity of symptoms, although this treatment is controversial. Other mood stabilizers and antiepileptics studied have
included valproic acid, carbamazepine, phenytoin, gabapentin, and lamotrigine.

None of these have consistently demonstrated significant benefit.

Antidepressants including tricyclics, selective serotonin reuptake inhibitors,
and serotonin-norepinephrine reuptake inhibitors have not been shown to have
any prophylactic effect either. Only
lithium had a reported response rate significantly higher than medical
abstention. However, it should be noted that none of the drug trials have been
double-blinded or placebo controlled. A Cochrane review could not come to a
conclusion for pharmacological recommendation because there was no study
qualified for the inclusion criteria.

 

Pearls/Take
Home Points:

Kleine-Levin Syndrome
(KLS) is often under-recognized and also misdiagnosed. When suspicion for KLS
is raised, a thorough clinical evaluation should be performed, including
detailed history from family members and a neurologic and psychiatric
examination. Additional studies may include PSG, EEG, drug screen, neuroimaging,
as well as serological and CSF studies to rule out alternative diagnoses as
clinically indicated.

Although
controversial, lithium is used as therapy for prevention. However, in spite
of the progress made in neuroscience, the search for the cause, genetic factors,
pathophysiology and drug treatment of KLS is still ongoing. There has
been no definite conclusion for pharmacological treatments for KLS, and studies
are difficult due to the rarity and complexity of the disease.