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• A CASE OF RECURRENT BICKERSTAFF BRAINSTEM ENCEPHALITIS
  Josanne Aquilina
  Published on: 13 April 2016

• Published on: 13 April 2016

  A CASE OF RECURRENT BICKERSTAFF BRAINSTEM ENCEPHALITIS

  • Josanne Aquilina, Consultant Neurologist
  • Other Contributors:
    • Maria Mallia, Stefania Chetcuti Zammit

  We read with great interest the review by Dr Shahrizaila and Prof Yuki on Bickerstaff brainstem encephalitis (BBE) and Fisher syndrome (FS) (1) and would like to refer in particular to the section about recurrent illnesses. As stated, FS and BBE are typically monophasic and recurrent episodes are rare. Although recurrent episodes have been described in the literature, after a thorough literature search, only one clear do...

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  We read with great interest the review by Dr Shahrizaila and Prof Yuki on Bickerstaff brainstem encephalitis (BBE) and Fisher syndrome (FS) (1) and would like to refer in particular to the section about recurrent illnesses. As stated, FS and BBE are typically monophasic and recurrent episodes are rare. Although recurrent episodes have been described in the literature, after a thorough literature search, only one clear documented case of recurrent BBE was found. (2) In this respect, we would like to present our case of recurrent Bickerstaff Brainstem Encephalitis. A 35 year old male patient presented with a one day history of progressively worsening double vision, numbness and paraesthesia of the hands and feet and unsteady gait. A week prior to the onset of these symptoms, he admitted to having had a diarrhoeal illness which had resolved completely. The patient insisted that these symptoms were identical to those preceding a "viral encephalitis" he had suffered ten years before. On examination at the time he was fully conscious. However he had complex ophthalmoplegia and a wide-based, ataxic gait. Tone and power were normal in all four limbs and he was areflexic. Interestingly, both plantar responses were extensor. Sensory examination was normal. Initial blood investigations, CT scan and MRI of the brain, and cerebrospinal fluid (CSF) analysis were all normal. A clinical diagnosis of FS was made and he was immediately started on treatment with intravenous immunoglobulins (IvIg). Several hours later, on the same day of admission, the patient developed rapid deterioration in his level of consciousness. He became drowsy and non-communicative, vomited incessantly, and developed fever. He adopted a decorticate posture with markedly increased tone in all four limbs, the upper limbs in flexion and the lower limb in extension. He was urgently transferred to the Intensive Therapy Unit (ITU), intubated and ventilated. At this point a clinical diagnosis of BBE was made. The presence of progressive symmetrical ophthalmoplegia and ataxia, decreased level of consciousness, pyramidal signs and normal peripheral power met the criteria (3) for this condition. Intravenous Methylprednisolone was added to the treatment regime. A repeat MRI of the brain was normal. EEG showed theta activity consistent with a non-specific encephalopathy. Nerve conduction studies showed an axonal sensori-motor peripheral neuropathy. A serum sample for anti GQ1b antibodies taken prior to commencing treatment with IvIg eventually confirmed the diagnosis with a strongly positive titre (224; normal range up to 30). C. jejuni antibodies were also positive (1:80; normal range <1:10). During the patient's stay in ITU, he showed no neurological improvement despite treatment. In addition he had persistent pyrexia and evidence of dysautonomia with sinus tachycardia and labile hypertension. An elective tracheostomy was performed and a decision to empirically perform plasma exchange was taken after two weeks. During the fourth week in intensive care, the patient gradually started to regain consciousness and continued to improve. He was eventually transferred to the neurology ward where he underwent intensive rehabilitation and recovered fully after a period of about six weeks. He was seen at the neurology out-patients clinic 3 months following discharge from hospital where he was found to have no neurological deficit. Discussion with the patient's family revealed that at 25 years of age, the patient had suffered an identical illness and was treated in a hospital in his home country. Review of the case notes showed that the patient had developed abdominal pain and diarrhoea for 48 hours. Days after this resolved he had developed speech disturbances, numbness over the trunk and upper limbs and ataxia. There was then deterioration in level of consciousness with decorticate posturing and respiratory insufficiency necessitating transfer to intensive care. An MRI of the brain had shown a possible hypointense area in the medial temporal lobe. CSF had revealed normal protein 0.33 g/l and normal cell count. EEG had shown a slow background with occasional high voltage signals. Apart from receiving Ceftriaxone and Acyclovir, the patient had also received IvIg and intravenous steroids. At the time the presumed diagnosis was that of a viral encephalitis. In retrospect this event was more probably the patient's first episode of BBE following a diarrhoeal illness. There have been cases of FS or Guillaine-Barre syndrome (GBS) followed by a second episode which involved clouding of consciousness. (4,5) However we consider our case to be remarkable because it was recurrent BBE. To our knowledge, there is only one other reported case of recurrent BBE. (2) This same case showed MRI changes, namely hyperintensity on T2 weighted images in the brainstem, with no contrast enhancement. Furthermore we would like to highlight two other issues. The first is that in our patient there was clear progression from a clinical diagnosis of FS to BBE within 24 hours, giving support to the proposed "anti-GQ 1b antibody syndrome" with the two conditions being at the ends of a clinical spectrum. Moreover, although the outcome of BBE is more often than not benign, the duration of unconsciousness in an immobile decorticate posture with severe dysautonomia can lead to potentially life-threatening complications of sepsis and thromboembolism. Therefore we feel the need to emphasise the importance of having evidenced based and well defined therapeutic options aimed at shortening as far as possible this period of unconsciousness and would appreciate any contributions from your readership relevant to this.

  References:

  1. Shahrizaila N, Yuki N. Bickerstaff brainstem encephalitis and Fischer syndrome: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry 2013; 84:576-583 2. Mond?jar RR, Santos JM, Villalba EF. MRI findings in a remitting- relapsing case of Bickerstaff encephalitis. Neuroradiology 2002; 44(5): 411-4. 3. Odaka M, Yuki N, Hirata K. Anti-GQ1b IgG antibody syndrome: clinical and immunological range. J Neurol Neurosurg Psychiatry 2001; 70(1): 50-5.

  4. Sharma V, Chan Y C, Ong, Teoh H L, Wilder- Smith E P. Bickerstaff's brainstem encephalitis: can it recur? Journal of Clinical Neuroscience 2006; 13(2): 277-9.

  5. DONG Hui-qing, LIU Zheng, TANG Yi, LU Yan, WANG Qi and JIA Jian- ping. Recurrent Fisher- Bickerstaff syndrome: report of a Chinese case. Chinese Medical Journal 2011; 124(17): 2786-2788.

  Conflict of Interest:

  None declared

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  Conflict of Interest:
  None declared.

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