Case Diagnosis: Unpredicted recovery from Bickerstaff’s Encephalitis facilitated by exoskeleton gait training.
Case Description or Program Description: A previously healthy 35-year-old gentleman presented to the emergency department with ascending paresthesia, dizziness, and unsteady gait for 4 days. Physical examination revealed weakness, areflexia, anisocoria, bidirectional nystagmus, positive vertical skew, and decreased visual acuity of the right eye. He was diagnosed with Guillain-Barre Syndrome type IV versus Bickerstaff Encephalitis. Lumbar puncture showed albumin cytologic dissociation. EMG showed severe, axonal sensory-motor polyneuropathy.
Setting: The patient was treated with 5 days of IVIG without improvement. He was found to have progressive weakness and worsening vital capacity. He was placed on mechanical ventilation for 9 days. Once extubated and despite the typically dismal prognosis for both diagnoses, the patient was admitted to an inpatient rehab facility.
Assessment/Results: The patient regained movement in his upper extremities with intensive rehabilitation including exoskeleton robotic gait training. He improved from requiring total assistance for mobility, and global weakness, to being mod-I on most ADLs, and ambulating short distances with a rolling walker and CGA. He was discharged to the community with assistance.
Discussion (relevance): Post-infectious demyelinating diseases are extensively described in the literature but none of them emphasized the role of rehabilitation, particular therapeutic approaches, or positive outcomes. A combination of symptoms involving the central and peripheral nervous system suggested an overlap of Guillain- Barre Syndrome and Bickerstaff Brainstem Encephalitis. The prognosis is usually poor. This case however demonstrates dramatic improvement attributed primarily to intensive multi-therapy inpatient rehabilitation with new techniques.
Conclusions: This case demonstrates that despite negative prognostic literature, individual patients can exceed medical expectations. New rehabilitation interventions challenge typical paradigms of who is appropriate for inpatient rehab and what outcomes to expect. This case study is an essential contribution to the literature as it demonstrates interventions that are novel to this diagnosis and have an untypical outcome.