Melissa Sun, DO, MS
Resident Physician/ Dr
Penn State PM&R
Hummelstown, Pennsylvania, United States
Swetha Sundaram, MS
Medical Student
Rowan-Virtua SOM
Voorhees, New Jersey, United States
Frank Anzelmi, MD
Neurohospitalist
Virtua Health
Voorhees, New Jersey, United States
Bickerstaff Brainstem Encephalitis (Anti-Gq1b Syndrome)
Case Description:
A 29-year-old female with no pertinent PMHx presented to the ED with blurry vision, generalized weakness, and ataxia for 1 week s/p mechanical fall with head trauma. She had associated symptoms of numbness and tingling to the bilateral UE and LE. Of note, she had a self-resolving cough 2-weeks prior. Pertinent FHx includes maternal multiple sclerosis. Pertinent physical exam findings included bilateral internuclear ophthalmoplegia and 4+/5 strength for bilateral hip flexion. CTH and CTA H/N were negative for acute intracranial abnormalities. MRI brain and MRI C/T-spine w/ and w/o showed no areas of contrast enhancements. IVIG was initiated. During the stay, she developed new-onset dysphagia and worsening symptoms leading to an LP which revealed pleocytosis and was ultimately upgraded to the ICU. Lab work revealed high titers of anti-Gq1b which confirmed the final diagnosis of Bickerstaff Brainstem Encephalitis. Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune encephalitis that was first described by Bickerstaff et al. in 1951 (1). This disease is part of the family of immune-mediated polyneuropathies, including Guillan-Barré syndrome (GBS) and Miller-Fisher Syndrome (MFS). BBE is differentiated from other demyelinating disorders due to its central nervous system involvement. The main symptom triad includes ataxia, ophthalmoplegia, and altered consciousness. Other associated symptoms include facial diplegia, babinski’s sign, pupillary abnormality, and bulbar palsy (3). The diagnosis is clinical and can be confirmed by positive serum antiganglioside antibodies (Anti-Gq1b) though not needed in diagnosis as it was only seen positive in 66% of diagnosed cases (2,3). Early involvement in physical therapy and inpatient acute rehabilitation can benefit the patient with the best outcome and return to function. This patient was transferred to an IPR and was discharged with a new functional status of modified independence for all ADLs, IADLs, and transfers.
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