PMID- 2335040
OWN - NLM
STAT- MEDLINE
DCOM- 19900613
LR  - 20191029
IS  - 0009-9155 (Print)
IS  - 0009-9155 (Linking)
VI  - 21
IP  - 2
DP  - 1990 Apr
TI  - Somatosensory evoked potentials in Hallervorden-Spatz-neuroaxonal-dystrophy 
      complex with dorsal column involvement.
PG  - 58-66
AB  - Two patients with a marked hypointensity of the globus pallidus on magnetic 
      resonance imaging (MRI), which is known to be diagnostic for Hallervorden-Spatz 
      disease (HSD), are presented. Patient 1 fell ill at about 10 years of age with 
      visual disturbance, spastic paraplegia and mild ataxia, while patient 2 was 
      affected during the second year of life with clinical features compatible with 
      infantile neuroaxonal dystrophy (INAD). The two patients had certain clinical 
      features in common; upper and lower motor neuron involvement, visual disturbance 
      secondary to optic nerve atrophy, and dorsal column dysfunction, the evidence of 
      which was seen from abnormal somatosensory evoked potentials (SEPs) obtained 
      after posterior tibial nerve stimulation. In both patients, electron microscopic 
      examination of the biopsied skin or sural nerve showed dystrophic axons, 
      spheroids, and involvement of the peripheral nerve was indicated. Sharing of 
      these clinical, pathological and MRI characteristics by the two patients supports 
      the view of Seitelberger, who regarded HSD and INAD as constituents of a single 
      disease entity, therefore the two patients were described as belonging to a 
      disease spectrum of "Hallervorden-Spatz-neuroaxonal-dystrophy complex (HS-ND)." 
      Sensory impairment has been a rare clinical feature in "HS-ND" complex, although 
      its existence is not inconceivable considering the usual affection of the dorsal 
      column/lemniscal pathway with spheroids. SEP was considered very useful in 
      disclosing this often unmanifested sensory disturbance in "HS-ND" complex.
FAU - Mutoh, K
AU  - Mutoh K
AD  - Department of Pediatrics, Faculty of Medicine, Kyoto University, Japan.
FAU - Okuno, T
AU  - Okuno T
FAU - Ito, M
AU  - Ito M
FAU - Mikawa, H
AU  - Mikawa H
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Clin Electroencephalogr
JT  - Clinical EEG (electroencephalography)
JID - 0236454
SB  - IM
MH  - Adult
MH  - Basal Ganglia Diseases/*diagnosis
MH  - Child
MH  - *Evoked Potentials, Somatosensory
MH  - Globus Pallidus/pathology
MH  - Humans
MH  - *Magnetic Resonance Imaging
MH  - Male
MH  - Neural Pathways/physiopathology
MH  - Pantothenate Kinase-Associated Neurodegeneration/*diagnosis/physiopathology
MH  - Spinal Cord/*physiopathology
EDAT- 1990/04/01 00:00
MHDA- 1990/04/01 00:01
CRDT- 1990/04/01 00:00
PHST- 1990/04/01 00:00 [pubmed]
PHST- 1990/04/01 00:01 [medline]
PHST- 1990/04/01 00:00 [entrez]
AID - 10.1177/155005949002100204 [doi]
PST - ppublish
SO  - Clin Electroencephalogr. 1990 Apr;21(2):58-66. doi: 10.1177/155005949002100204.