Central pontine signal changes in Wilson's disease: distinct MRI morphology and sequential changes with de-coppering therapy.
OBJECTIVE
Reports of central pontine myelinolysis (CPM)-like changes in Wilson's disease (WD) and its sequential changes are exceptional. The aim was to study the MRI characteristics of CPM-like changes in WD and the serial changes.


METHODS
Among the 121 patients of WD, twenty (M:F:9:11, age at onset: 14.2 +/- 4.6 years) had features similar to CPM. All had progressive neuropsychiatric form of WD. All except five were on de-coppering treatment. None had acute deterioration or hepatic failure. Ten patients underwent repeat studies.


RESULTS
Twenty patients with CPM-like changes manifested with characteristic phenotype of WD. Three distinct patterns of CPM-like changes were observed: (a) characteristic round shape -7, (b) "bisected" -9, and (c) "trisected" -4. Only one had signal changes suggesting extra-pontine myelinolysis. All patients had contiguous involvement of midbrain. Serial MRI evaluation in 10 patients, at mean interval period of 17.4 +/- 13.2 months, revealed complete reversal in one, partial improvement in five, and no change in three. Clinical and MRI improvement occurred pari passu, except in one.


CONCLUSIONS
CPM-like changes in WD are perhaps under-recognized and are distinct from the commonly known "osmotic demyelination." It is potentially reversible similar to other MRI features of WD.
Sinha
Sanjib
S
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India. sanjib_sinha@nimhans.kar.nic.in
Taly
Arun B
AB
Ravishankar
Shivashankar
S
Prashanth
Lingappa K
LK
Vasudev
Mandapati K
MK
D000293:Adolescent; D000328:Adult; D001252:Astringents / Q000627:therapeutic use; D002614:Chelating Agents / Q000627:therapeutic use*; D016009:Chi-Square Distribution; D002648:Child; D018450:Disease Progression; D005260:Female; D006305:Health Status Indicators; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007194:India; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D010641:Phenotype; D011149:Pons / Q000473:pathology*; D011379:Prognosis; D011446:Prospective Studies; D019287:Zinc Sulfate / Q000627:therapeutic use
D001252:Astringents; D002614:Chelating Agents; D019287:Zinc Sulfate; D010396:Penicillamine