XXXX channel antibody-associated @entity75 : a potentially immunotherapy-responsive form of @entity14204 .

@entity1 @entity972 @entity410 @entity198 @entity75 @entity73 @entity1372 @entity9137 @entity2730 @entity12296 @entity911 @entity194 @entity8231 @entity230 @entity2414 @entity14204
@entity1 presenting with subacute @entity2730 are frequently seen in acute neurological practice. Amongst the differential diagnoses, herpes simplex @entity198 , @entity9137 and @entity14204 should be considered. @entity14204 is typically a @entity8231 with a poor prognosis; thus, identifying those @entity1 with potentially reversible symptoms is important. Voltage-gated @entity972 channel antibodies (VGKC-Ab) have recently been reported in three cases of reversible @entity14204 . Here we review the clinical, immunological and neuropsychological features of 10 @entity1 (nine male, one female; age range 44-79 years), eight of whom were identified in two centres over a period of 15 months. The @entity1 presented with 1-52 week histories of @entity2414 , @entity911 and @entity410 . Low plasma @entity1372 concentrations, initially resistant to treatment, were present in eight out of 10. Brain MRI at onset showed signal change in the medial temporal lobes in eight out of 10 cases. Paraneoplastic antibodies were negative, but VGKC-Ab ranged from 450 to 5128 pM (neurological and healthy controls <100 pM). CSF oligoclonal bands were found in only one, but bands matched with those in the serum were found in six other @entity1 . VGKC-Abs in the CSF, tested in five individuals, varied between <1 and 10% of serum values. Only one @entity1 had @entity12296 , which was excluded by electromyography in seven of the others. Formal neuropsychology testing showed @entity2414 , with sparing of general intellect in all but two @entity1 , and of nominal functions in all but one. Variable regimes of @entity230 , plasma exchange and intravenous immunoglobulin were associated with variable falls in serum VGKC-Abs, to values between 2 and 88% of the initial values, together with marked improvement of neuropsychological functioning in six @entity1 , slight improvement in three and none in one. The improvement in neuropsychological functioning in seven @entity1 correlated broadly with the fall in antibodies. However, varying degrees of @entity194 and residual @entity73 were common. Over the same period, only one paraneoplastic case of @entity14204 was identified between the two main centres. Thus, VGKC-Ab-associated @entity75 is a relatively common form of autoimmune, non-paraneoplastic, potentially treatable @entity198 that can be diagnosed by a serological test. Establishing the frequency of this new syndrome, the full range of clinical presentations and means of early recognition, and optimal immunotherapy, should now be the aim.
