Bickerstaff’s Brainstem Encephalitis (BBE) is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. This condition has been named Bickerstaff’s brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the . Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by Edwin Bickerstaff in It may also affect the .

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Bickerstaff brainstem encephalitis

Copyright American Medical Association. The patient had transient and very frequent episodes of brainstem dysfunction during the recovery phase.

Frequent episodes of transient brainstem dysfunction occurred in our patient during recovery, possibly due to ephaptic transmission. In a bfainstem review, Odaka et al 2 defined BBE as a disorder with progressive ophthalmoplegia and ataxia and with features suggesting a central nervous system process, such as disturbed consciousness or hyperreflexia.

In this report, we describe brainatem patient who had BBE with a number of remarkable features. The diagnosis was made quickly, partly through the use of magnetic resonance imaging MRI studies.

Methylprednisolone sodium succinate and prednisone were used as the only BBE treatment and led to a complete recovery, stressing the importance of early disease recognition and aggressive treatment—even in elderly patients such as ours. Of special interest was the development of very brief transient bikcerstaff of brainstem dysfunction that occurred hundreds of times a day during the recovery phase.

An year-old woman was admitted on April 2,because of progressively slurred speech, diplopia, clumsiness, and unsteady walking.

One month before admission, the patient had developed a severe nonproductive cough that was diagnosed as interstitial pneumonitis and treated with moxifloxacin hydrochloride. The respiratory problems initially improved, but then the cough significantly worsened before the onset of neurological problems.

Three days before admission, the patient developed dysarthria followed by diplopia, clumsiness, and ataxia. On admission, the patient was afebrile with a mildly disturbed mental state and dysarthria. She exhibited incomplete horizontal eye movements with no ptosis or pupillary abnormalities. Results of the motor examination were unremarkable except for a questionable right Babinski sign and unsteady gait. The patient had dysmetria and slowed rapid alternating movements in the upper extremities.

Results of the sensory examination were normal. A chest radiograph showed an increase in interstitial markings and small right-sided effusion. A brain MRI showed increased signal on fluid-attenuated inversion recovery image sequences, with gadolinium enhancement in the brainstem Figure.

A magnetic resonance angiogram showed no abnormalities. Blood study results showed no elevation of antibodies to neurotropic viruses, Brucellaor Mycobacterium tuberculosis.

Results of serological studies on a variety of respiratory pathogens showed no elevations that would suggest a recent infection. A tentative diagnosis of BBE was made, and the patient was transferred to the neurological intensive care unit. The patient quickly developed dysphagia and hyperactive reflexes, as well as cardiac arrhythmias and signs of acute kidney injury.

On April 5, acyclovir administration was discontinued and mechanical ventilation for airway protection and hemodynamic stabilization was started. On April 6, the patient was noted to have bilateral Babinski signs with unsustained ankle bickerwtaff she became more difficult to arouse, with extensor posturing on the right side.


A second MRI showed extension of the brainstem involvement without new lesions. A cervical spine MRI showed no abnormalities. An electroencephalogram showed marked generalized slowing in the theta range, suggestive of an encephalopathy. Results of a second lumbar brainstemm on April 7 were normal. Rbainstem April 10, the patient was more responsive and a tetraparesis became apparent. The examination on April 11 showed horizontal gaze abnormalities, ptosis, seventh nerve palsies, and increased strength.

Over the next several days, the patient improved in mental state, ptosis, eye movements, and renal function and was therefore extubated. Prednisone therapy was begun. The patient’s speech became clearer and her reflexes more normal. A few days later, she noticed very frequent, stereotypic episodes lasting 1 to 10 seconds and occurring throughout the day; these episodes consisted of transient diplopia encephwlitis by dysarthria if talking and gait imbalance if walking.

The episodes increased in frequency so that they occurred up to hundreds of times a day. The patient was witnessed to have multiple episodes of paroxysmal worsening of slurred speech and ataxia with diplopia. Between episodes, results of an examination showed minimal dysarthria, full eye movements, no ptosis, a questionable right Babinski sign, slight clumsiness, and a slow gait with a encephalitus Romberg sign.

Treatment for Fisher syndrome, Bickerstaff’s brain stem encephalitis and related disorders

Electroencephalograms and electrocardiograms showed no abnormalities during the episodes. Magnetic resonance imaging showed a decrease in the abnormal signal in the brainstem with no evidence of ecephalitis stroke FigureD and E. When seen by one of us R.

In addition to the episodes, the patient had minimal slurring of words when she first began to speak and a slight imbalance when she began to walk. Over the next few weeks, the episodes disappeared enceephalitis the patient returned to her normal baseline condition. Therefore, patients are generally classified as having BBE rather than MFS enecphalitis there is evidence of corticospinal tract abnormalities, a disturbed state of consciousness, or a significant brainstem lesion on MRI bickerstff at autopsy.

At times, there is an overlap between BBE and GBS, which prompted Odaka et al 2 to classify some patients with BBE who had symmetrical flaccid limb weakness as having BBE associated with GBS; electrophysiological study results of these patients can suggest a motor axonal neuropathy, presumably representing an acute motor axonal neuropathy variant of GBS. In our case, the presence of Babinski signs, a disturbed state of consciousness, and the dramatic MRI signal abnormalities in the brainstem indicated a diagnosis of BBE.

The patient’s weakness presumably resulted from the extensive brainstem lesion involving the corticospinal tracts and was not related to an associated GBS.

The age of the patient was not at all typical for patients with BBE and contrasts with previously presented cases. A remarkable feature of our patient was the presence of brief paroxysmal episodes of brainstem dysfunction that appeared after recovery of much of her neurological function.

During these episodes, the patient exhibited transient ataxia, diplopia, and dysarthria—deficits that had been seen on a continuous basis earlier in the disease course. For several weeks, the episodes occurred hundreds of times a day, lasting 1 to 10 seconds. They were not related to a seizure phenomenon or cardiovascular abnormality. Episodes of sensory disturbance are not infrequent in demyelinating processes such as multiple sclerosis.


Episodes that involve more complex abnormalities are not common but have been described. The episodes of brainstem dysfunction in our patient resembled similar events reported by Andermann et al 10 and Espir et al.

Our patient’s episodes exhibited several brainstem abnormalities suggesting mass synchronous firing as a result of ephaptic transmission see Smith and McDonald 12 for a review. In this condition, neuronal excitability can spread laterally to adjacent axons that may have an abnormally lowered threshold for firing as a result of demyelination. Interestingly, axons that are experimentally demyelinated develop spontaneous activity with a lowered threshold for firing 1 week or more after the insult, possibly explaining the onset of these episodes during the patient’s recovery.

Orphanet: Bickerstaff brainstem encephalitis

The dramatic MRI abnormalities in our patient assisted us in making an early diagnosis, which allowed prompt treatment of BBE. Various treatments of BBE have been used in the past, including corticosteroids, intravenous immunoglobulin, and plasmapheresis. Our patient was treated with corticosteroids, which seemed to have a positive therapeutic effect.

Additional treatment with intravenous immunoglobulin and plasmapheresis was not used because of concerns about our patient’s renal and hemodynamic status. Despite the fact that this elderly woman had significant neurological deficits and serious medical problems, she eventually returned to her baseline status, highlighting the importance of aggressive, supportive treatment in what can be a completely reversible process.

Study concept and design: Roos, Goldenberg, and Badruddin. Analysis and interpretation of data: Roos, Soliven, and Baron. Drafting of the manuscript: Critical revision of the manuscript for important intellectual content: Roos, Soliven, Goldenberg, Badruddin, and Baron. Privacy Policy Terms of Use. Report of a case. Sign in to access your subscriptions Sign in to your personal account.

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