Kleine–Levin syndrome: a disease in its own right?

Clinico-Pathological Correlations of the Most Common Neurodegenerative Dementias

“…Some studies have found a characteristic distribution of neuritic plaques and neurofibrillary tangles, with antero-posterior gradient in occipito-parietal regions (lower density in primary visual cortex), and relative sparing of frontal cortex (Levine et al, 1993; Hof et al, 1997). When comparing PCA and typical AD phenotype, it was found that the former had higher density of neuritic plaques and neurofibrillary tangles in visual association cortex and the later had higher density of lesions in hippocampus and subiculum, but there were no differences in other cortical areas (Tang-Wai et al, 2004). Clinical progression may disclose clues to underlying pathology: development of episodic memory impairment and involvement of other cognitive domains suggests AD; visual hallucinations, delusions, and parkinsonism may indicate DLB; asymmetric parkinsonism and ideomotor apraxia may suggest corticobasal degeneration, CBD; rapid progression of global disability, myoclonus, and cortical blindness with Anton syndrome suggest prion disease (namely the Heidenhain variant of Creutzfeldt–Jakob disease).…”

“…Clinical progression may disclose clues to underlying pathology: development of episodic memory impairment and involvement of other cognitive domains suggests AD; visual hallucinations, delusions, and parkinsonism may indicate DLB; asymmetric parkinsonism and ideomotor apraxia may suggest corticobasal degeneration, CBD; rapid progression of global disability, myoclonus, and cortical blindness with Anton syndrome suggest prion disease (namely the Heidenhain variant of Creutzfeldt–Jakob disease). It should be noted, however, that despite the etiological diversity of PCA, in over 75% of cases, AD pathology was shown (Renner et al, 2004; Tang-Wai et al, 2004). …”