Bickerstaff brainstem encephalitis

Bickerstaff's brainstem encephalitis (BBE) is a rare post-infectious neurological disease characterized by the association of external ophthalmoplegia, ataxia, lower limb arreflexia, extensor plantar response and disturbance of consciousness (drowsiness, stupor or coma).

Input patient's signs and symptoms

Narrow down the case reports

Total: 29 (papers)

(per page)

Matched Phenotype Gene Mutation MeSH

Rank (Similarity)	PMID (PMCID)
10 (4.0%)	30031375 (6054855)	Miller Fisher syndrome, Bickerstaff brainstem encephalitis and Guillain-Barre syndrome overlap with persistent non-demyelinating conduction blocks: a case report. Puma A, Benoit J, Sacconi S, Uncini A. BMC Neurol. 2018;18(1):101. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia
		CST7

		Encephalitis Guillain-Barre Syndrome Homo sapiens Male Middle Aged Miller Fisher Syndrome
10 (4.0%)	29878345	Headache and Ophthalmoparesis: Case Report of an "Atypical" Incomplete Miller-Fisher Syndrome. Moreno-Ajona D, Irimia P, Fernandez-Matarrubia M. Headache. 2018;58(5):746-749. [ Show abstract ] [ Hide abstract ]
		Ophthalmoparesis


		Autoantibodies Females Gangliosides Headache Homo sapiens Immunoglobulins Middle Aged Miller Fisher Syndrome Ophthalmoplegia
10 (4.0%)	29792178 (5966867)	Anti-ganglioside complex antibody profiles in a recurrent complicated case of GQ1b-seronegative miller fisher syndrome and Bickerstaff brainstem encephalitis: a case report. Ito H, Hatanaka Y, Fukami Y, Harada Y, Kobayashi R, Okada H, Uchibori A, Chiba A, Okuda S. BMC Neurol. 2018;18(1):72. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia


		Adult Autoantibodies Brain Stem Encephalitis Gangliosides Guillain-Barre Syndrome Homo sapiens Male Miller Fisher Syndrome Phenotype
10 (4.0%)	29489680 (5851734)	Miller-Fisher syndrome complicated by Bickerstaff brainstem encephalitis: A case report. Jing C, Wang Z, Chu C, Dong M, Lin W. Medicine (Baltimore). 2018;97(9):e9824. [ Show abstract ] [ Hide abstract ]
		Blurred vision


		Brain Stem Differential Diagnosis Diffusion Magnetic Resonance Imaging Encephalitis Females Homo sapiens Middle Aged Miller Fisher Syndrome
10 (4.0%)	28637936	A case of Bickerstaff brainstem encephalitis with transient reflex myoclonus. Takaki R, Nagasaka T, Suwa Y, Tsuchiya M, Kho K, Takiyama Y. Rinsho Shinkeigaku. 2017;57(7):395-398. [ Show abstract ] [ Hide abstract ]
		Dysarthria


		Adult Autoantibodies Brain Stem Encephalitis Females Gangliosides Homo sapiens Mycoplasma Infections Myoclonus Myography Pulse Therapy, Drug Time Factors
10 (4.0%)	27540012	An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. Cuneo GL, Grazzini I, Guadagni M, Venturini E, Bianchi A. Neuroradiol J. 2016;29(5):396-9. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia


		Antigens, CD1 Ataxia Autoantibodies Brain Stem Encephalitis Females Gangliosides Homo sapiens Image Processing, Computer-Assisted Magnetic Resonance Imaging
10 (4.0%)	27406055	[Bickerstaff brainstem encephalitis after upper respiratory infection]. Locht LJ, Blaabjerg M. Ugeskr Laeger. 2016;178(27):. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia


		Brain Stem Encephalitis Females Guillain-Barre Syndrome Homo sapiens Middle Aged Respiratory Tract Infections
10 (4.0%)	25890931	Progressive subacute Miller-Fisher syndrome successfully treated with plasmapheresis. Ejma M, Waliszewska-Prosol M, Hofman A, Budrewicz S, Podemski R, Bilinska M, Koszewicz M. Neurol Neurochir Pol. 2015;49(2):137-8. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia


		Autoantibodies Gangliosides Homo sapiens Male Miller Fisher Syndrome Plasma Exchange Plasmapheresis
10 (4.0%)	24853856	Successful treatment of refractory Guillain-Barre syndrome with alemtuzumab in a patient with chronic lymphocytic leukemia. Tzachanis D, Hamdan A, Uhlmann EJ, Joyce RM. Acta Haematol. 2014;132(2):240-3. [ Show abstract ] [ Hide abstract ]
		Leukemia


		Antibodies, Monoclonal, Humanized Antibodies, Monoclonal, Murine-Derived Antigens, Neoplasm Antineoplastic Combined Chemotherapy Protocols Autoantibodies Autoantigens CD Antigens Chronic Lymphocytic Leukemia Combined Modality Therapy Consciousness Disorders Gangliosides Glycoproteins Guillain-Barre Syndrome Homo sapiens Male Miller Fisher Syndrome Nitrogen Mustard Compounds Plasmapheresis Remission Induction Virus Activation
10 (4.0%)	24763765	Inefficacy of antipsychotics in treatment of delirium and agitation in two cases of bickerstaff brainstem encephalitis. Alam A, Puri NV. J Neuropsychiatry Clin Neurosci. 2014;26(2):176-8. [ Show abstract ] [ Hide abstract ]
		Agitation


		Antipsychotic Agents Brain Stem Delirium Electroencephalography Encephalitis Homo sapiens Magnetic Resonance Imaging Male Middle Aged Psychomotor Agitation

Phenotype(s) retrieved from Orphanet

Total: 0

HPO ID	Term	Frequency

Phenotype(s) retrieved from case reports

Total: 11

HPO ID	Term	# of case reports
HP:0000602	Ophthalmoplegia	2
HP:0001251	Ataxia	2
HP:0000819	Diabetes mellitus	1
HP:0001284	Areflexia	1
HP:0001297	Stroke	1
HP:0001336	Myoclonus	1
HP:0001909	Leukemia	1
HP:0002015	Dysphagia	1
HP:0002090	Pneumonia	1
HP:0002329	Drowsiness	1
HP:0002664	Neoplasm	1

Causative gene(s) retrieved from Orphanet

Total: 0

Gene Symbol	Gene Name	Entrez Gene ID