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Similar findings were confirmed in a larger NT1 population, whereas in childhood NT1 at disease onset a particular facial involvement (“cataplectic facies”) occurred spontaneously in the context of a complex movement disorder that vanished during disease course. psychogenic non-cataplectic attack) when the complaints are not objectively confirmed.įew studies documented cataplexy, showing three sequential phases: “initial” with eye closure, knees buckling, and twitches “falling” with smile interruption, jaw sagging, rhythmic postural lapses, and fall and “atonic” laying on the ground. A difficult differential diagnosis is conversion disorder (functional neurological symptom disorder ) with mixed symptoms, to be clinically classified as pseudonarcolepsy and pseudocataplexy (i.e. Up to 46 per cent of non-narcoleptic subjects report “episodes of muscle weakness with various emotions or athletic activities”, a phenomenon resembling “weakness with laughter” in the general population. This clinical variability challenges the differential diagnosis. However, a larger phenotypic spectrum exists encompassing between- and within-patients variability of the role of triggers, hypotonia distribution (partial and generalized), frequency, and duration of the attacks.

Cataplexy “needs to be established based on the clinical interview alone because patients are rarely examined during an attack”. Cataplexy is pathognomonic for NT1 and is characterized, as per consensus criteria, by episodes of brief, symmetrical, loss of muscle tone with retained consciousness precipitated by strong emotions. Narcolepsy type 1 (NT1) is a central hypersomnia linked to the loss of hypothalamic hypocretinergic neurons. Attacks of episodic fall/collapse should be documented because video-recorded attacks provide reliable information useful for phenotyping ambiguous cases. An abrupt facial involvement of hypotonia interrupting laughter behavior marked cataplexy versus psudocataplexy in video-documented attacks of patients with narcolepsy type 1 and functional neurological symptoms. Cataplexy assessment is exclusively based on clinical interview, possibly challenging the correct diagnosis of narcolepsy.