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Table 2 Distribution of neuronal loss with gliosis

From: Clinicopathologic features of two unrelated autopsied patients with Charcot-Marie-Tooth disease carrying MFN2 gene mutation

 

Patient 1

Patient 2

Cerebrum

  

  Frontal cortex

–

–

  Motor cortex

–

–

  Temporal cortex

–

–

  Parietal cortex

–

–

  Occipital cortex (striate cortex)

–/++

–/+

  White matter*

–

–

Subcortical area

  

  mmon (CA1/subiculum)

–/–

–/–

  Amygdaloid nucleus

–

–

  Caudate nucleus/putamen

–/–

–/ N.A.**

  Globus pallidus i/e

–/–

–/–

  Thalamus

–

–

  Subthalamic nucleus

–

–

  Lateral geniculate body

+++

N.A.**

Brainstem

  

  Substantia nigra

–

–

  Pontine nucleus

–

–

  Cranial motor nerve nuclei

(IV/V motor/VI/VII/XII)

–/–/–/N.A./–

–/–/N.A./–/–

  Ambiguus nucleus

–

–

  Dorsal vagal nucleus

–

–

  Inferior olivary nucleus

–

–

  Reticular formation

–

–

  Vestibular/cochlear nuclei

–/N.A.

–/N.A.

  Gracile/cuneate nuclei

+++/++

+++/++

Cerebellum

  

  Cortex/white matter*

–/+

–/+

  Dentate nucleus

–

–

  Spinal cord

  

  Anterior horn (C/Th/L/S)

+/++/++/++

+/+/++/++

  Clarke’s nucleus

+++

+++

  Intermediolateral nucleus

–

–

  Lateral corticospinal tract*

+

+

  Spinocerebellar tract (Vent./Dors.)*

–/+

–/+

  Anterior funiculus (C/Th/L/S)*

–/–/–/–

++/+ ± /–

  Posterior funiculus (gracile/cuneate)*

++/+

++/+

Peripheral nerve

  

  Dorsal root ganglion (C/L)

+/++

+/+

  Anterior nerve root (C/L)*

+/++

+/N.A.

  Posterior nerve root (C/L)*

+/++

+/N.A

  Sural nerve*

+++

+++

  Sympathetic ganglion

–

–

Other

  

  Optic nerve*

+++

++

  1. Neuronal loss with gliosis, +++: Severe, ++: Moderate, +: Mild, –: None, N.A. Not available. i/e: Internal/external segment; Vent. Ventral; Dors. Dorsal
  2. *Loss of myelinated fibers. **Not available due to putaminal hemorrhage