• Clinical and Experimental Pediatrics
• /
• v.57 no.12
• /
• pp.542-545
• /
• 2014

Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.

• Annals of Clinical Neurophysiology
• /
• v.11 no.1
• /
• pp.33-36
• /
• 2009

Bickerstaff's brainstem encephalitis (BBE) is an autoimmune central nervous system disorder. It can occur in more limited forms and may overlap with Guillain-Barr$\acute{e}$ syndrome (GBS). A 49-year-old female presented with rapidly progressive paralytic ileus, urinary retention, deep drowsiness, ophthalmoplegia, dysarthria, ataxia, quadriparesis and hyporeflexia after viral meningitis. She was diagnosed as BBE with GBS and treated with immunoglobulin. She was completely recovered after 1 month. It is a rare case of BBE overlapping with GBS presenting with severe paralytic ileus.

• Annals of Clinical Neurophysiology
• /
• v.8 no.1
• /
• pp.94-97
• /
• 2006

Bickerstaff's brainstem encephalitis (BBE) is a rare disorder with acute ophthalmoparesis, ataxia, consciousness disturbance, and pyramidal signs of suspected autoimmune origin. A-62-year-old woman developed acute diplopia, dysarthria, gaze-paretic nystagmus and gait ataxia. Her mental status fell subsequently into stupor. Brain MRI and nerve conduction study showed no significantly abnormal findings. Electroencephalography revealed diffuse low voltage slowings. After treating with intravenous immunoglobulin, she demonstrated rapid clinical recovery. This case suggests that immunoglobulin can be an alternative option in BBE treatment, especially when plasmapheresis and corticosteroids are difficult or contraindicated.

• Clinical and Experimental Pediatrics
• /
• v.57 no.10
• /
• pp.457-460
• /
• 2014

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barr$\acute{e}$ syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.

• Annals of Clinical Neurophysiology
• /
• v.15 no.1
• /
• pp.30-33
• /
• 2013

• Clinical and Experimental Pediatrics
• /
• v.53 no.4
• /
• pp.607-611
• /
• 2010

Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic methods: brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins.

• Annals of Clinical Neurophysiology
• /
• v.8 no.1
• /
• pp.78-80
• /
• 2006

Miller-Fisher syndrome, Guillain-Barre syndrome with ophthalmoplegia, Bickerstaff s brainstem encephalitis and acute ophthalmoplegia share some clinical features, and common anti-GQ1b IgG antibody and these are introduced as anti-GQ1b antibody syndrome. These syndromes mostly present with paralysis of extraocular muscles and internal ophthalmoplegia rarely occurs. We report a case of acute isolated bilateral internal ophthalmoplegia associated with anti-GQ1b IgG antibody.