Kleine-Levin + Kluver-Bucy + Moyamoya + Niemann Pick Type C

Question 1

what is Kleine-Levin syndrome

Answer 1

Kleine–Levin Syndrome (KLS) is a rare neuropsychiatric disorder characterized by persistent episodic hypersomnia, hyperphagia, cognitive impairment, and mood changes. Individuals can also experience hypersexuality, personality changes, and behavioural changes.

Question 2

what is Kluver-Bucy syndrome

Answer 2

neuropsych syndrome

caused by LESIONS of the BILATERAL TEMPORAL LOBES

hyperorality, sexual hyperactivity, changes in dietary behaviour, hypermetamorphosis, visual agnosia, placidity

Question 3

what are the most common risk factors for Kluver-Bucy syndrome

Answer 3

TBI

stroke

HSV encephalitis in children

but can be cause by any disease process that causes acute bitemporal injury or by progressive neurodegenerative disorders that affect the bilateral anterior temporal lobes

Question 4

what is moyamoya disease

Answer 4

Moyamoya Disease (Japanese: “puff of smoke”) is a cerebrovascular disorder characterized by progressive unilateral or bilateral stenosis of the distal internal carotid arteries (often extending to the anterior and middle cerebral arteries). Up to a quarter of patients may present with heterogenous psychiatric symptoms that may be misdiagnosed as a behavioural (especially in children), mood, or psychotic disorder. The etiology of Moyamoya is unknown.

Question 5

what is the core clinical feature of moyamoya disease

Answer 5

ischemia secondary to hypoperfusion, which can cause repeated transient ischemic attacks (TIAs) or stroke in the cerebral cortex (e.g. - frontal, parietal, and temporal lobes)

The disruption of the anterior circulation can result in hemiparesis, speech disturbance, and hemisensory impairments

Question 6

what are some psychiatric symptoms of moyamoya disease

Answer 6

Depression or anxiety in assessment of new focal neurological symptoms is more common.

Transient ischemic attacks may be mistaken for anxiety and panic disorder.

Psychiatric symptoms in moyamoya disease is heterogeneous, and affects up to 25% of patients.

In adults, refractory depression and psychosis with new focal neurological deficits may be signs of moyamoya.[1]