Dados do Trabalho

Título

BICKERSTAFF ENCEPHALITIS – CASE REPORT

Apresentação do caso único

A healthy 4-year 3-month-old male patient presented with dysarthria, plegia of the lower limbs, gait ataxia, divergent strabismus, and vomiting. He was taken to the emergency room already exhibiting decreased consciousness, which had progressed over two days since the onset of symptoms. His parents reported flu-like symptoms six days prior and a poliomyelitis vaccination ten days before. A cranial CT scan showed no abnormalities. A lumbar puncture revealed normal cerebrospinal fluid analysis, and PCR for herpes virus was negative. He was transferred to the intensive care unit, requiring orotracheal intubation due to decreased consciousness. The patient developed cranial nerve involvement, including anisocoria, absence of pupillary reflex, bilateral divergent strabismus, bilateral ptosis, facial hypomimia, and limb areflexia. Based on the symptoms and clinical suspicion of a variant of Guillain-Barré Syndrome (GBS) and considering the possibility of Acute Disseminated Encephalomyelitis (ADEM), the patient was treated with methylprednisolone and intravenous human immunoglobulin. An electroencephalogram showed moderately disorganized baseline activity without paroxysms. A new lumbar puncture performed 13 days after symptom onset revealed protein-cytological dissociation: cellularity of 8 cells/mm³ and protein of 90mg/dL. Anti-ganglioside antibody IgG (anti-GQ1b), tested 14 days after symptom onset, was negative. The patient developed symptoms of dysautonomia. Electroneuromyography three months after symptom onset indicated sensory and motor polyneuropathy, axonal, symmetrical, with pronounced ongoing denervation activity, but signs of reinnervation in proximal muscles of the upper limb. The patient required a tracheostomy, which was successfully removed after approximately three months. The patient showed improvement, regaining the ability to walk, though lower limb areflexia persisted.

Discussão

Bickerstaff encephalitis is a variant of GBS, or acute immune-mediated polyneuropathies. It presents as brainstem encephalitis characterized by encephalopathy and hyperreflexia, although hyporeflexia, ophthalmoplegia, and ataxia may also occur. It is associated with anti-GQ1b antibodies and can respond to intravenous immunoglobulin and plasmapheresis.

Comentários finais

This is a rare condition in the general population, particularly in the pediatric age group. Due to its high morbidity and mortality, proper suspicion and treatment are crucial for changing the patient's prognosis.

Referências

Dimachkie MM, Barohn RJ. Guillain-Barré syndrome and variants. Neurologic clinics. 2013 May 1;31(2):491-510.

Hiew FL, Ramlan R, Viswanathan S, Puvanarajah S. Guillain-Barré Syndrome, variants & forms fruste: Reclassification with new criteria. Clinical Neurology and Neurosurgery. 2017 Jul 1;158:114-8.

Palavras Chave

Birckenstaf; Guillain Barre Syndrome; Encephalitis