A.

History of the disease

Bickerstaff brainstem encephalitis is an immune disorder of unknown etiology, first
described by Edwin Bickerstaff in 1951. It may also affect the peripheral nervous system, and
has features in common with both Miller Fisher syndrome and Guillain–Barré syndrome
Characteristics:
 Acute, progressive cranial nerve dysfunction.

 Associated cerebellar ataxia.

 Coma

B. Pertinent findings in the history of present illness and physical diagnosis

The patient diagnosed with Bickerstaff brainstem encephalitis exhibited fever, headache
along with dysarthria and vomiting for 2 days. The patient was noted to be drowsy but oriented
to time and place. Upon ocular examination, the patient exhibits bilateral gaze-evoked nystagmus
and mild restriction of eye movement and bilaterally impaired cerebellar signs which are the
failed finger-to-nose test, dysdiadochokinesia and heel-to-shin-test. The clinical background of
the presenting symptoms of the patients points to brain stem encephalitis.

Epidemiology
 It is very rare and mostly reported in adults; however, cases affecting children have also
been reported.
 Very often it follows an illness, and an association with certain infections, including
cytomegalovirus, Campylobacter jejuni, typhoid fever and Mycoplasma pneumoniae, has
been documented
Presentation
 Acute diplopia.
 Ataxia.
 Pyramidal tract paralysis.
 Disturbance of consciousness.
 Headache is common.
 Progressive, symmetrical ophthalmoplegia, ataxia and either disturbance of
consciousness or hyperreflexia.
 Facial palsy, extensor plantar reflex, pupillary abnormality, nystagmus and bulbar palsy.
 It may result in apnoea and a reversible brain death picture.
Differential diagnosis
 Multiple sclerosis.
 Behçet's disease.
 Lyme disease.
 Progressive multifocal leukoencephalopathy.
 Sarcoidosis.
 Whipple's disease.
  Listeria rhombencephalitis.
 Vasculitis due to systemic lupus erythematosus (SLE).
 Acute disseminated encephalomyelitis

Treatment
A. Pharmacological

Generic name: Intravenous immunoglobulin (IVIG)

Brand name: IBivigam, Carimune,Flebogamma
Indication: to reduce the effects of some inflammatory conditions that involve the immune
system, also known as autoimmune diseases.

B. Non pharmacological

 Physical therapy and Occupational therapy for other symptoms of BBE

 Speech therapy if patient is with bulbar palsy

1. Discuss the anatomy involved in the case

a. Trace the pathway from the brainstem to the extraocular muscles

The brainstem pathways for horizontal eye movements start from the abducens nucleus as the
horizontal gaze center taking signal from the PPRF. These pathways continue to the ipsilateral
abducens nerve (cranial nerve VI) and the contralateral oculomotor nerve (cranial nerve III)
through the MLF and end in conjugate horizontal eye movement in the ipsilateral direction to the
side of the abducens nucleus.

b. Enumerate the cranial nerve nuclei with the brainstem, and briefly discus their
pathway and terminal function

The extraocular muscles are innervated by lower motor neurons that form three cranial nerves:
the abducens, the trochlear, and the oculomotor. The abducens nerve (cranial nerve VI) exits the
brainstem from the pons-medullary junction and innervates the lateral rectus muscle. The
trochlear nerve (IV) exits from the caudal portion of the midbrain and supplies the superior
oblique muscle. In distinction to all other cranial nerves, the trochlear nerve exits from the dorsal
surface of the brainstem and crosses the midline to innervate the superior oblique muscle on the
contralateral side. The oculomotor nerve (III), which exits from the rostral midbrain near the
cerebral peduncle, supplies all the rest of the extraocular muscles. Although the oculomotor
nerve governs several different muscles, each receives its innervation from a separate group of
lower motor neurons within the third nerve nucleus.
 2. Briefly touch on the neurophysiology of muscle movement

For a contraction to occur there must first be a stimulation of the muscle in the form of an
impulse (action potential) from a motor neuron (nerve that connects to muscle). 

The individual motor neuron plus the muscle fibers it stimulates, is called a motor unit.  The
motor end plate (also known as the neuromuscular junction) is the junction of the motor neurons
axon and the muscle fibers it stimulates.

When an impulse reaches the muscle fibers of a motor unit, it stimulates a reaction in each
sarcomere between the actin and myosin filaments.  This reaction results in the start of a
contraction of the muscle. 

The cortex of the vermis influences the movements of the long axis of the body, namely, the
neck, the shoulders, the thorax. the abdomen, and the hips

Immediately lateral to the vermis is so-called intermediate zone of the cerebellar hemisphere.
This area has been shown to control the muscles of the distal part of the limbs, specially the
hands and feet

Lateral zone of each cerebellar hemisphere appears to be concerned with the planning of
sequential movements of the entire body and is involve in the conscious assessment of
movement

3. What other diagnostic tool might be helpful in this case?

A. Radiologic

MRI
It transiently involves the brainstem and basal ganglia and is characterized on MRI by regions of
high T2 signal with little - if any - enhancement. Typically, these regions also demonstrate some
minor restricted diffusion.

Brain Anatomy (MRI showing coronal view)

MRI with Bickerstaff Brainstem Encephalitis.

Image A and D- showing coronal View

- image shows hyperintense in brainstem region, as you can see in the T2

Coronal. (2 images in the left)

Image B, C, E, F- showing axial flair (perform only in the MRI Head)

Take note:

T2: fluid and fat appear as bright color

T1: Fat and fluid appear as hypointense or dark
Protocol they use in performing MRI head patients with possible Bickerstaff
brainstem Encephalitis is T1 and T1 with DWI or Diffusion weighted image.
Reason: In this protocol doctors will easily see if there are changes in the
appearance showing bright side in the brainstem.

Reason why MRI best modality for Bickerstaff Brainstem Encephalitis-

Because of the DWI- Diffusion Weighted Image. If there is a presence of
inflammation- there is a restriction that will show the changes in the
appearance in the image and will show the bright side.

B. Laboratory

What value does this test offer to the diagnosis and treatment?
Diagnosis is based on the clinical findings, patient history, cerebrospinal fluid (CSF) analysis
(revealing raised protein levels), detection of anti-GQ1b IgG antibodies (not present in all
patients), MRI studies (revealing high-intensity abnormalities in the posterior fossa, white matter
or thalami) and neurophysiological examinations (electroencephalogram and electromyography
indicative of central nervous system and predominantly axonal involvement).

Laboratory test: Bacterial and Viral Culture with sample CSF

- This may rule out the specific organism and correlate its radiologic results to the BBE
Example of bacteria and virus are that may cause the BBE are the following: Cytomegalovirus.
Campylobacter jejuni and Mycoplasma pneuomoniae. BBE is associated with the presence of the
antiganglioside antibody, anti-GQ1b
Serum anti-GQ1b IgG antibody test (increased result around 91%)
- Patient with BBE
 external ophatlmoplegia
 ataxia
These findings together with the common autoantibody (anti-GQ1b IgG) suggest that a common
autoimmune mechanism functions in the pathogenesis of these illnesses. In BBE , acute
ophthalmoparesis are closely related. This is supported by the immunological findings. The term
"anti-GQ1b IgG antibody syndrome" is not intended to be used as a clinical diagnosis, but
recognition of this syndrome is useful for understanding the aetiological relation among the
various illnesses