Premium
Central Pontine Signal Changes in Wilson's Disease: Distinct MRI Morphology and Sequential Changes with De‐Coppering Therapy
Author(s) -
Sinha Sanjib,
Taly Arun B.,
Ravishankar Shivashankar,
Prashanth Lingappa K.,
Vasudev Mandapati K.
Publication year - 2007
Publication title -
journal of neuroimaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.822
H-Index - 64
eISSN - 1552-6569
pISSN - 1051-2284
DOI - 10.1111/j.1552-6569.2007.00120.x
Subject(s) - central pontine myelinolysis , medicine , magnetic resonance imaging , radiology
BACKGROUND AND PURPOSE 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.