PMID- 8289437
OWN - NLM
STAT- MEDLINE
DCOM- 19940224
LR  - 20080408
IS  - 0023-2513 (Print)
IS  - 0023-2513 (Linking)
VI  - 39
IP  - 4
DP  - 1993 Aug
TI  - Infantile neuroaxonal dystrophy--immunohistochemical and ultrastructural studies 
      on the central and peripheral nervous systems in infantile neuroaxonal dystrophy.
PG  - 133-46
AB  - We performed pathological studies on the central and peripheral nervous systems 
      of cases with infantile neuroaxonal dystrophy (INAD). Numerous spheroid bodies in 
      the central and peripheral nervous systems, were seen and divided into large 
      spheroid bodies (LSB) and small spheroid bodies (SSB) photo-microscopically. LSB 
      had a relation to some specific neurons with weak expression of neuron specific 
      enolase, neurofilament and chromogranin using PAP method. SSB showed a relation 
      to the axon without immunohistochemical expression of neuron specific enolase, 
      neurofilament, glial fibrillary acidic protein, myelin basic protein, 
      chromogranin, S 100 protein or antitrypsin. LSB were prominent in the posterior 
      column, gracile nucleus, cuneate nucleus, and the tegmentum of the midbrain and 
      the pons associated with neuronal loss and gliosis. SSB were observed in the 
      thalamus, basal ganglia and the cerebral cortex. The cerebellum was sclerotic 
      with few microtubule-like structures disposed in a dense network in association 
      with degenerated mitochondria. Similar changes were observed in the sural nerves, 
      autonomic nerve endings in the skin, and the nerve plexus of the digestive tract. 
      Although INAD is a generalized neurodegenerative disease, it is suggested that 
      the primary disorder might occur in the neurons and axons of the sensory tracts.
FAU - Itoh, K
AU  - Itoh K
AD  - Department of Pathology, Kobe University School of Medicine, Japan.
FAU - Negishi, H
AU  - Negishi H
FAU - Obayashi, C
AU  - Obayashi C
FAU - Hayashi, Y
AU  - Hayashi Y
FAU - Hanioka, K
AU  - Hanioka K
FAU - Imai, Y
AU  - Imai Y
FAU - Itoh, H
AU  - Itoh H
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Japan
TA  - Kobe J Med Sci
JT  - The Kobe journal of medical sciences
JID - 0413531
SB  - IM
MH  - Axons/*ultrastructure
MH  - Humans
MH  - Infant
MH  - Male
MH  - Microscopy, Electron
MH  - Muscular Dystrophies/*pathology
MH  - Nervous System Diseases/*pathology
MH  - Peripheral Nervous System Diseases/pathology
EDAT- 1993/08/01 00:00
MHDA- 1993/08/01 00:01
CRDT- 1993/08/01 00:00
PHST- 1993/08/01 00:00 [pubmed]
PHST- 1993/08/01 00:01 [medline]
PHST- 1993/08/01 00:00 [entrez]
PST - ppublish
SO  - Kobe J Med Sci. 1993 Aug;39(4):133-46.