The other BSE

Abstract

Brainstem encephalitis has long proved complex and cyptic, but the waters are beginning to clear. Six decades ago, Edwin Bickerstaff (1921–2008), writing from Birmingham with Professor Philip Cloake (1890–1969), described three cases of ‘mesencephalitis and rhombencephalitis’ (Bickerstaff and Cloak, 1951). Each individual presented with a brainstem oculomotor disturbance, other cranial neuropathies and ataxia (with variable other signs); two had a hypercellular cerebrospinal fluid. The patients recovered well; and though lamenting the lack of pathological characterization, the authors considered ‘their clinical features and course were so remarkably similar that it seemed highly probable that the same or a closely related pathological process was involved’. Six years later, Bickerstaff published eight cases of what he now designated ‘brainstem encephalitis’ (Bickerstaff, 1957); others later came to attach Bickerstaff's name to the disorder. The waters were, however, already becoming rather muddied: only one case had come to autopsy, and no brainstem inflammation was apparent (Bickerstaff, 1957). What is more, four of these eight cases of ophthalmoplegia and ataxia had areflexia (one also with extensor plantar responses); and 1 year earlier Charles Miller Fisher had described three cases of ‘an unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia)’ (Fisher, 1956), which of course also came to bear his name. There followed a long period of uncertainty as to whether Fisher and Bickerstaff syndromes were one and the same, and of central or peripheral origin; only in the last few years has (mainly) immunological characterization begun to resolve these matters. This brief neuro-history offers a backdrop to last year's fascinating description in Brain of a new brainstem inflammatory syndrome. Sean Pittock, Jan Debruyn, Brian Weinshenker and Mark Keegan and colleagues, from the Mayo Clinic (USA) and from Ghent University Hospital (Belgium), beautifully and succinctly (if perhaps not euphoniously) described ‘CLIPPERS’ (Pittock et …