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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