![]() ![]() Each has ataxia with cerebellar incoordination of the limbs. Five children, eight young adults and five older subjects all developed drowsiness and acute progressive ophthalmoplegia, often both external and internal, and associated with facial and bulbar weakness. With Edwin Bickerstaff as co-author, Amir Al-Din and colleagues now aim to show that the syndrome of acute ophthalmoplegia, ataxia and areflexia is distinct from the Guillain–Barré syndrome and the pathology does indeed affect the brainstem.Įighteen cases are reported. After the appearance of Charles Miller Fisher's paper in 1956 ( New Engl J Med 1956: 255 57–65) in which, reporting three cases and building on the suggestion by James Collier (1870–1935) that the Guillain–Barré syndrome may show external ophthalmoplegia with or without limb paralysis and in which Dr Fisher emphasizes the increase in cerebrospinal fluid protein without pleocytosis, the style of case series has been to designate this syndrome as a variant of acute post-infectious polyneuritis. The typical form is characterized by acute progressive ophthalmoplegia, involvement of other cranial nerves, ataxia and hyporeflexia, no significant motor or sensory deficit in the limbs and usually with full recovery. The team from Birmingham opens their new analysis of this disorder by stating unambiguously that ‘brainstem encephalitis includes the syndrome of ophthalmoplegia, ataxia and hyporeflexia’. ![]() In 1957, Edwin Bickerstaff (1920–2007) followed up on three cases of mesencephalitis and rhomboencephalitis he had reported 6 years earlier with Philip Cloake (1890–1969), describing as a definitive syndrome eight patients with acute symmetrical ophthalmoplegia, other cranial nerve palsies and ataxia ( Br Med J 1957: 1 1384–1387). ![]()
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