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)
1 (35.3%)	24463996	A case report of overlapping Bickerstaff brainstem encephalitis and Guillain-Barre syndrome. Feng J, Fu X, Xie W, Lv X, Zhang Y, Meng H. Neuro Endocrinol Lett. 2013;34(7):601-5. [ Show abstract ] [ Hide abstract ]
		Facial paralysis


		Autoantibodies Brain Stem Encephalitis Gangliosides Guillain-Barre Syndrome Homo sapiens Magnetic Resonance Imaging Male X-Ray Computed Tomography Young Adult
1 (35.3%)	23268979	[A case of Fisher syndrome with multiple cranial neuropathy and abnormal EEG findings]. Ochi M, Shinohara N, Kamogawa K, Okada Y, Nagai T, Taguchi K, Takita R, Igase M, Kohara K, Miki T. Nihon Ronen Igakkai Zasshi. 2012;49(3):367-71. [ Show abstract ] [ Hide abstract ]
		Bilateral facial palsy


		Electroencephalography Females Homo sapiens Miller Fisher Syndrome
1 (35.3%)	22306424	Unusual manifestations in two cases of necrotizing myopathy associated with SRP-antibodies. Hanisch F, Muller T, Stoltenburg G, Zierz S. Clin Neurol Neurosurg. 2012;114(7):1104-6. [ Show abstract ] [ Hide abstract ]
		Necrotizing myopathy Bilateral facial palsy


		Adenocarcinoma Aspiration Pneumonia Autoantibodies Biopsy Fatal Outcome Guillain-Barre Syndrome Homo sapiens Kidney Neoplasm Male Middle Aged Miller Fisher Syndrome Myopathy Necrosis Neoplasm Metastasis Neurologic Examination Ophthalmoplegia Paraneoplastic Syndromes, Nervous System Signal Recognition Particle X-Ray Computed Tomography
1 (35.3%)	20120214	IVIG- responsive multiple cranial neuropathy: a pharyngo-facial variant of Guillain-Barre syndrome. Unal-Cevik I, Onal MZ, Odabasi Z, Tan E. Acta Neurol Belg. 2009;109(4):317-21. [ Show abstract ] [ Hide abstract ]
		Facial paralysis


		Aged, 80 and over Females Guillain-Barre Syndrome Homo sapiens
1 (35.3%)	18717185	[A case of Bickerstaff brainstem encephalitis concomitant with axonal Guillain-Barre syndrome and ballism successfully treated with intravenous immunoglobulin treatment]. Makino F, Kojima Y, Inoue M, Satoi H, Kanda M, Shibasaki H, Kusunoki S. Rinsho Shinkeigaku. 2008;48(7):501-4. [ Show abstract ] [ Hide abstract ]
		Facial paralysis
		CSF2

		Adult Brain Stem Encephalitis Gangliosides Guillain-Barre Syndrome Homo sapiens Male Miller Fisher Syndrome
1 (35.3%)	12451613	Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems. Ogawara K, Kuwabara S, Yuki N. Muscle Nerve. 2002;26(6):845-9. [ Show abstract ] [ Hide abstract ]
		Facial diplegia


		Acoustic Evoked Brain Stem Potentials Adult Brain Stem Electroencephalography Encephalitis Females Gangliosides H-Reflex Homo sapiens Miller Fisher Syndrome Peripheral Nervous System Plasmapheresis Sleep Stages Somatosensory Evoked Potentials
7 (17.5%)	29953036	A Case Report of Overlapping Miller Fisher Syndrome, Guillain-Barre Syndrome, and the Bickerstaff Brainstem Encephalitis. Chae CS, Kwon KM, Lee JS, Kim YH. Neurologist. 2018;23(4):128-130. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia Muscle weakness


		Brain Stem Encephalitis Females Guillain-Barre Syndrome Homo sapiens Miller Fisher Syndrome Syndrome
7 (17.5%)	22989900	[Case of Bickerstaff brainstem encephalitis associated with spindle coma and decorticate posture]. Shimozono K, Shimono K, Kusunoki S. Rinsho Shinkeigaku. 2012;52(9):656-9. [ Show abstract ] [ Hide abstract ]
		Hyperhidrosis Difficulty walking


		Adult Autoantibodies Biological Markers Brain Stem Comatose Combination Drug Therapy Decerebrate State Electroencephalography Encephalitis Gangliosides Homo sapiens Male
7 (17.5%)	9294325	[A case report of Bickerstaff brainstem encephalitis with attention to electronystagmographic findings]. Sugihara R, Fujimoto S, Ueyama H, Kumamoto T, Tsuda T. Rinsho Shinkeigaku. 1997;37(5):388-92. [ Show abstract ] [ Hide abstract ]
		Nystagmus Muscle weakness


		Adult Brain Stem Caloric Tests Electronystagmography Encephalitis Females Homo sapiens Pursuit, Smooth
10 (4.0%)	30502045	Bickerstaff Brainstem Encephalitis and overlapping Guillain-Barre syndrome in children: Report of two cases and review of the literature. Michev A, Musso P, Foiadelli T, Trabatti C, Lozza A, Franciotta D, Simoncelli AM, Savasta S. Eur J Paediatr Neurol. 2019;23(1):43-52. [ Show abstract ] [ Hide abstract ]
		Ophthalmoplegia


		Brain Stem Child Electroencephalography Encephalitis Females Guillain-Barre Syndrome Homo sapiens Magnetic Resonance Imaging Male Neuroimaging Neurologic Examination

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