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Table 4 Distribution and severity of neurodegenerative changes in patients with BIBD

From: Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration

 

Cases with chorea

Cases without chorea

 

1

2

3

4

5

6

Brain weight (g)

800

1190

880

1140

940

1230

Cortical atrophy

F, T(base)

F(tip)

F&T(base)

F&T(base)

F,T(base)

T(tip)

Frontal cortex

+++

+++

++

+++

+++

+++

Cingulate gyrus

+++

+++

+++

+++

+++

+++

Temporal cortex

++

+

++

++

+++

+++

Hippocampus

+++

+

+++

+++

na

+++

Amygdala

+++

+

na

+++

+++

+++

Caudate nucleus

+++

+++

+++

+++

+++

+++

Putamen

+++

+

+++

++

+++

+++

Globus pallidus

+++

±

++

++

++

++

Thalamus

+++

±

±

++

+++

+

Subthalamic nucleus

±

±

±

±

±

±

Nucleus basalis of Meynert

+

-

±

±

±

+

Cerebellar dentate nucleus

±

-

±

±

±

+

Red nucleus

±

±

±

na

na

±

Substantia nigra

+++

+

+++

++

+++

+++

Locus coeruleus

+++

-

±

±

++

++

Pontine nucleus

±

±

±

±

±

±

Dorsal vagal nucleus

±

±

±

na

±

±

Hypoglossal nucleus

±

-

±

±

±

±

Inferior olivary nucleus

±

±

±

±

+

+

Spinal cord

-

-

na

na

na

±

  1. Data for cases 2 through 6 were assembled from previously published materials [15, 29]. F, frontal lobe; T, temporal lobe; F(tip), frontal tip; F(base), frontal base, T(tip), temporal tip; T(base), temporal base. Rating for the severity of degeneration: -, no degeneration; ±, no neuronal loss but gliosis; +, slight neuronal loss and gliosis; ++, moderate neuronal loss and gliosis; +++, severe neuronal loss and gliosis. Degeneration in the corticospinal tract: +, present; -, absent. na, tissue not available