Silence after the storm; a case report on Bickerstaff Brainstem encephalitis

Öz

A pediatric case with Bickerstaff’s brainstem encephalitis (BBE), which is a very rare monophasic post-infectious condition characterized by central nervous system involvement, unconsciousness, ophthalmoplegia and ataxia, is presented. Twelve years old patient was brought with difficulty in eye movements and ataxia. On the second day, she became agitated and lethargic, and then bulbar palsy and whole body paralysis developed. Upper motor neuron involvement was evident. Routine biochemical parameters and serologic tests, cranial magnetic resonance imaging, lumbar puncture, autoimmune, paraneoplastic, and electrophysiological studies were evaluated. All were normal except for the encephalopathic first electroencephalography (EEG) and the EEG repeated on the 25th day was reported to be normal. Anti-ganglioside antibody, anti-GQ1b was found positive. Intravenous immunoglobulin (IVIG) started on the fourth day. A very rapid improvement was seen in the first week of IVIG treatment. She was able to walk in the second week. She was completely normal in 3 months. Although seven years passed, our patient has not had any relapse or neurological deficit. We presented a case diagnosed as BBE treated successfully with single dose intravenous Immunoglobulin. We wanted to emphasize that BBE has a good prognosis even though it is an acutely developing severe condition.  

Anahtar Kelimeler

• Ataxia
• Brainstem
• Encephalitis
• Intravenous immunoglobulin

Fırtına Sonrası Sessizlik; Bickerstaff Beyin Sapı Ensefalitli Bir Olgu

Öz

A pediatric case with Bickerstaff’s brainstem encephalitis (BBE), which is a very rare monophasic post-infectious condition characterized by central nervous system involvement, unconsciousness, ophthalmoplegia and ataxia, is presented. Twelve years old patient was brought with difficulty in eye movements and ataxia. On the second day, she became agitated and lethargic, and then bulbar palsy and whole body paralysis developed. Upper motor neuron involvement was evident. Routine biochemical parameters and serologic tests, cranial magnetic resonance imaging, lumbar puncture, autoimmune, paraneoplastic, and electrophysiological studies were evaluated. All were normal except for the encephalopathic first electroencephalography (EEG) and the EEG repeated on the 25th day was reported to be normal. Anti-ganglioside antibody, anti-GQ1b was found positive. Intravenous immunoglobulin (IVIG) started on the fourth day. A very rapid improvement was seen in the first week of IVIG treatment. She was able to walk in the second week. She was completely normal in 3 months. Although seven years passed, our patient has not had any relapse or neurological deficit. We presented a case diagnosed as BBE treated successfully with single dose intravenous Immunoglobulin. We wanted to emphasize that BBE has a good prognosis even though it is an acutely developing severe condition.

Anahtar Kelimeler

• Ataksi
• Bickerstaff beyin sapı
• Ensefaliti
• İntravenous immunoglobulin

Kaynakça

1. Yuki N, Sato S, Tsuji S, Hozumi I, Miyatake T . An immunologic abnormality common to Bickerstaff’s brain stem encephalitis and Fisher’s syndrome. J Neurol Sci 1993;118: 83-7.
2. Odaka M, Yuki N, Hirata K. Anti-GQ1b IgG antibody syndrome: clinical and immunological range. J Neurol Neurosurg Psychiatry 2001;70: 50-5.
3. Shahrizaila N, Yuki N. Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome J Neurol Neurosurg Psychiatry 2013;84:576-583
4. Santoro JD, Lazzareschi DV, Campen CJ, Van Haren KP Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series Journal of Neurology 2018; 265:141-150