Association of reversible splenial lesion syndrome (RESLES) with Anti-VGKC autoantibody syndrome: a case report

Abstract

A 50-year-old male presented with complaints of fatigue, confusion, and memory problems. Neurological evaluation revealed altered cognition, unsteady gait, ataxia, dysmetria, and weakness. MRI of the brain was initially unremarkable. Over several days, the patient experienced improvement of symptoms and a follow-up MRI revealed a small lesion in the splenium of the corpus callosum seen on diffusion weighted and T2 sequences. The patient was discovered to have elevated anti-voltage gated potassium channel serum autoantibodies. Follow-up MRI revealed resolution of the splenial lesion. The patient was treated with intravenous immune globulin, and improved back to his pre-treatment baseline. We believe this to be the first case of a reversible splenial lesion syndrome as a manifestation of the anti-voltage gated potassium channel autoantibody syndrome, and propose a pathophysiologic mechanism.

Reversible Splenial Lesion Syndrome with Some Novel Causes and Clinical Manifestations

“…Antiepileptic drug toxicity and associated changes in salt homeostasis and transhemispheric seizure propagation are other suspected mechanisms (30,31). Cell-cytokine interactions lead to massively elevated extracellular glutamate levels, and this phenomenon is thought to be important in Disturbed consciousness (24,40), somnolence (40)(41)(42), confusion (15,21,40,43), memory problems (15,21,42), disorientated (41,43,44), apathy (40,45), mental abnormalities (46), delirious behavior (24), delirium (47), agraphia (48), ideomotor apraxia (49), alien hand syndrome (49), autotopagnosia (49) Disturbed consciousness (7,9,12,18,(56)(57)(58), lethargy (25), somnolence (25,26,59), confusion (26), cognitive impairment (26), behative impairment (26), behavioral disorders (26), hallucination (25), delirium (25,57), disorientated …”

Reversible splenial lesion syndrome (RESLES) coinciding with cerebral venous thrombosis: a report of two cases

“…DWI showed restricted diffusion with decreased ADC map values. 1 , 6 In certain cases, the lesion shows complete involvement of the splenium on diffusion-weighted image, otherwise known as the ‘boomerang sign’. The pathophysiology of RESLES is obscure.…”

N‐methyl‐d‐aspartate receptor encephalitis with a reversible splenial lesion

“…A potential association with autoimmune processes has been documented in cases including autoimmune thyroid disease [8]. Co-occurrence of a reversible splenial lesion and autoantibodies against voltage-gated potassium channels was described in one patient [9]. The authors discussed the potential causal action of voltage-gated potassium channel autoantibodies resulting in a change in potassium concentration within the myelin sheaths.…”