Brainstem Encephalitis. The Role of Imaging in Diagnosis

Sotoudeh, Houman; Razaei, Ali; Saadatpour, Zahra; Gaddamanugu, Siddhartha; Choudhary, Gagandeep; Shafaat, Omid; Singhal, Aparna

doi:10.1067/j.cpradiol.2020.09.004

Cited by 13 publications

(10 citation statements)

References 53 publications

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“…Discreet, longitudinally extensive T2 hyperintensities in the pontine and medullary tegmentum, extending into the cervical cord (Figure , A-C), without mass effect, without enhancement has been reported in paraneoplastic disorders. 3 Typical features of demyelinating disease and CLIPPERs (pontine perivascular enhancement) were absent. 4 CSF studies revealed 37 white blood cells/μL (normal, <5/μL, 92%), lymphocyte-predominant, protein of 59 (normal, ≤35 mg/dL), normal glucose, normal IgG index and synthesis rate, and 4 CSF-exclusive oligoclonal bands (normal, <2).…”

Section: Sectionmentioning

confidence: 98%

“…Opsoclonus (with or without myoclonus) is rarely encountered beyond immune-mediated etiologies. 3 A comprehensive evaluation including blood tests, MRI imaging, CSF analysis, and EEG was undertaken. General tests (complete blood count with differential, electrolytes, fasting glucose, C-reactive protein, liver function tests, urinalysis, and chest x-ray) showed normal results.…”

Section: Sectionmentioning

confidence: 99%

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Clinical Reasoning: A 43-Year-Old Man With Subacute Onset of Vision Disturbances, Jaw Spasms, and Balance and Sleep Difficulties

et al. 2022

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A brainstem syndrome is recognizable in patients presenting with a combination of visual disturbances, incoordination, gait problems, speech and swallowing difficulties, and new-onset sleep symptomatology. Brainstem disorders of subacute onset (onset and progression with accumulation of disabling deficits in 6–12 weeks) are generally of autoimmune, infectious, inflammatory, or infiltrative neoplastic cause. An autoimmune or infectious brainstem disorder may be referred to as brainstem encephalitis or rhombencephalitis. We describe a patient with paraneoplastic autoimmune rhombencephalitis, in whom diagnostic clues included the following: diverse visual and sleep symptoms, trismus, and choking in the history; see-saw nystagmus, opsoclonus, dysarthria, jaw dystonia, and episodic laryngospasm on examination; subtle but longitudinal and nonenhancing T2 MRI abnormalities in the brainstem and upper cervical cord; and oligoclonal bands in the CSF. His movement disorder–specific neural IgG profile revealed ANNA-2 (anti-Ri) and KLHL-11-IgG. Both are biomarkers of paraneoplastic brainstem encephalitis. KLCHL-11-IgG has been reported to accompany germ cell tumors, which was found in a solitary metastasis to the left inguinal lymph node in our patient, along with an atrophic left testis. Multidisciplinary treatment (autoimmune neurology, sleep medicine, ophthalmology, and physiatry) led to significant clinical improvements. This case provides a framework for the evaluation of patients with subacute-onset brainstem syndromes and the investigation and management of those with paraneoplastic and other autoimmune diseases.

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Section: Sectionmentioning

confidence: 98%

Section: Sectionmentioning

confidence: 99%

Clinical Reasoning: A 43-Year-Old Man With Subacute Onset of Vision Disturbances, Jaw Spasms, and Balance and Sleep Difficulties

et al. 2022

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“…Following differential diagnosis, among others, should be taken in close consideration when seeing a patient with brainstem encephalitis: NMOSD, Behçet disease, Bickerstaff encephalitis, Listeria encephalitis and CLIPPERS [ 12 ]. The combination of brainstem encephalitis with LETM in our patient, lead towards the differential diagnosis of NMOSD and MOGAD.…”

Section: Discussionmentioning

confidence: 99%

MOG antibody associated disease (MOGAD) presenting with extensive brain stem encephalitis: A case report

et al. 2022

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“…MRI is important when ruling out alternative aetiologies of brainstem encephalitis. 15 16 Serial MRI studies have shown hyperintense signal changes on T2-weighted images in BBE, though MRI abnormality is still uncommon, reported in 11%–30% of BBE cases. 2 4 Our case report showed a completely normal MRI with and without contrast of the brain and spinal cord.…”

Section: Discussionmentioning

confidence: 99%

Bickerstaff’s brainstem encephalitis mimicking herpetic encephalomyelitis in a liver transplant patient with anti-GQ1b antibodies

Bhatia

Canepa

Notarianni³

2022

BMJ Case Rep

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A woman in her late 70s with a history of liver transplant presented with ophthalmoplegia, ataxia, areflexia, positive Babinski’s sign and reduced consciousness. This followed an antecedent illness in the form of a herpes zoster infection. MRI of the brain/spinal cord, cerebrospinal fluid analysis with viral PCR and routine blood tests were normal, and tacrolimus neurotoxicity was ruled out. Serum anti-GQ1b antibodies were positive. A diagnosis of Bickerstaff’s brainstem encephalitis was made, forming part of the continuum that involves Miller-Fisher syndrome, entitled the ‘anti-GQ1b syndrome’. Complete recovery ensued without intravenous immunoglobulins or plasma exchange. The role of monitoring anti-ganglioside pattern change to predict or confirm disease recurrence and disease severity is further discussed.

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Brainstem Encephalitis. The Role of Imaging in Diagnosis

Cited by 13 publications

References 53 publications

Clinical Reasoning: A 43-Year-Old Man With Subacute Onset of Vision Disturbances, Jaw Spasms, and Balance and Sleep Difficulties

Clinical Reasoning: A 43-Year-Old Man With Subacute Onset of Vision Disturbances, Jaw Spasms, and Balance and Sleep Difficulties

MOG antibody associated disease (MOGAD) presenting with extensive brain stem encephalitis: A case report

Bickerstaff’s brainstem encephalitis mimicking herpetic encephalomyelitis in a liver transplant patient with anti-GQ1b antibodies

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