Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Abstract

ObjectiveArea postrema (AP) syndrome (defined as: nausea and/or emesis and/or singultus at onset of brainstem dysfunction) comprises complex pathophysiologic mechanisms triggered by different entities. The first objective was to assess the frequency of AP syndrome as a clinical feature in brainstem encephalitis (BE). Finding an especially high prevalence of AP syndrome in Bickerstaff brainstem encephalitis (BBE), we also analyzed the frequency of AP syndrome in other autoimmune diseases with anti‐ganglioside antibodies (Guillain–Barré syndrome (GBS) and its variants).MethodsWe systematically evaluated the prevalence of AP syndrome in BE in all patients treated at our university hospital during a 15‐year period. In a second step, BBE patients were compared to GBS and Miller Fisher syndrome (MFS) patients as clinical subtypes of a disease continuum without brainstem dysfunction.ResultsWe found AP syndrome in 8 of 21 BE patients, including 3 of 7 BBE and in 4 of 112 GBS/MFS patients. AP syndrome was as a frequent but under‐recognized feature of BE with a significant impact on patients’ well being.InterpretationManifestation of AP syndrome in BBE but also in GBS and its subtypes point toward a role of autoimmune antibodies that should be investigated in future studies. Considerable misdiagnosis or nonrecognition complicates diagnostic and therapeutic management. Therefore, AP syndrome should be considered in any episode of otherwise unexplained nausea, emesis, or singultus.

Area postrema syndrome: a neurological presentation of nausea, vomiting and hiccups

“…1 Rarely in adults, external mass effect (eg, tumours) can also affect this area. 6 Although the presence of anti-SSA is associated with serum AQP4-IgG positivity in the Asian population, it was negative in our case. 7 Interestingly, only 14% of patients presenting with APS are.…”

Isolated Area Postrema Syndrome Preceding the Diagnosis of Giant Cell Arteritis

“…NMOSD is the main diagnosis, and there are very few reports in the literature about other inflammatory diseases causing area postrema syndrome such as Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) 8 and Bickerstaff encephalitis. 9 …”

“…NMOSD is the main diagnosis, and there are very few reports in the literature about other inflammatory diseases causing area postrema syndrome such as Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIP-PERS) 8 and Bickerstaff encephalitis. 9 A possible mechanism for the association of area postrema syndrome and giant cell arteritis is unknown, but it is known that giant cell arteritis can cause acute ischemic stroke by the involvement of the intracranial arteries, sometimes with multiple territory mimicking embolic stroke. 10 The pathologic processes suggested by a stroke secondary to giant cell arteritis are high-grade stenosis and occlusion.…”

Postvaccination acute disseminated encephalomyelitis with <i>area postrema</i> syndrome and quasi benign paroxysmal positional vertigo: a case report

“…Безусловно, САР не специфичен для NMOSD. Теоретически, любое структурное поражение АР может вызвать САР, что подтверждается обширным кругом заболеваний, при которых описан данный синдром: ишемический инсульт, рассеянный склероз, энцефалит Биккерстаффа, объёмные образования IV желудочка, хроническое лимфоцитарное воспаление с периваскулярным накоплением контрастного вещества в варолиевом мосту, реагирующее на терапию глюкокортикоидами (синдром CLIPPERS), болезнь Александера [13][14][15][16][17][18][19][20].…”