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Table 2 Distribution of neuronal storage material and PAS-positive granules in the central nervous system. Screening for neuronal inclusions was performed on sections stained with cresyl violet luxol fast blue (LFB) and sudan black (SB) or under ultraviolet light excitation for emission of autofluorescence (AF)

From: Homozygous TBC1 domain-containing kinase (TBCK) mutation causes a novel lysosomal storage disease – a new type of neuronal ceroid lipofuscinosis (CLN15)?

Region Neuronal storage material PAS + granules
LFB/SB AF
Cerebrum
 Basal ganglia Neocortex I – IV ++ ++
Neocortex V - VI +++ +++ ++
Nucleus caudatus (+) +
Putamen ++ (+)
Claustrum +++ +++
Thalamus ++ +++
 Hippocampus CA 1 ++ +++ +
CA 2–4 + ++ +
Corpus geniculatum laterale +++ +++ n. a.
 Cerebellum Cortex    
 Purkinje cells
 Granule cells
 Subcortical white matter +++
Nucleus dentatus (+) ++ +++
Brain stem
 Mesencephalon Substantia nigra (+) ++
Nucleus oculomotorius (+) n. a.
 Pons Pontine nuclei (+) + +++
Raphe nuclei (+) + +++
 Medulla oblongata Olivary nuclei +++ +++ (+)
Nucleus nervus vagus +++ +++
Nucleus nervus hypoglossus ++
  1. Note the distinctly higher sensitivity of detection with autofluorescence. n. a., no available PAS-stained section or paraffin-embedded tissue