A.

Introduction
A pleasant afternoon to our dear doctors and future doctors!
In behalf of section E, Im ____________ and I will be presenting to you our
opinion regarding our very first case for this semester. So lets begin
Based on the manifestations of the patient, one question that we should ask to
ourselves is - is there a neurologic problem? Our answer is yes because the patient
presented to us with a focal neurologic problem in the form of cranial nerve deficits,
incoordination, and poor equilibrium which is located on the left side. Focal neurologic
deficit can be classified as to its course as acute, subacute and chronic. Based on the
course of the patients disease, we classified it as chronic, which ranges from weeks to
months (months to years). From focal to chronic, this leads to our consideration, which
is a neoplasm. The next question is where is the location of the neoplasm? Since the
patient presented with signs of acoustic nerve involvement manifested as left-sided
hearing loss with associated tinnitus, numbness of the left side of the face , and upon
physical examination the it was revealed that the patient veers to the left on tandem gait
and the rhombergs sign was positive, the location of the tumor might probably be in the
left cerebello-pontine angle.

B. Pathophysiology
Cerebellopontine angle syndrome is commonly caused by the following in order
of frequency: vestibular schwannoma (60-90%), meningioma (3-7%), and epidermoid
cyst (2-4%). These usually grow gradually and usually present with almost the same
manifestation.
Tumors, as we all know, result from genetic alterations that are passed down to
the progeny tumor cells. These genetic changes allow excessive and unregulated
proliferation that becomes autonomous. This leads to tumor progression and growth
which may eventually cause mass effect. This mass effect or external pressure on the
adjacent nerve causes a decrease in blood flow thru vasa nevorum. This local ischemia
causes a decrease in glucose and oxygen supply which cause cessation of ATP
production. Since the Na-K ATPase pump is primarily dependent on ATP, this results to
an inability to maintain proper ionic gradient. This subsequently leads to loss of
neuronal excitability, massive release of glutamate, and eventual necrosis.

[picture: Orientation of normal anatomy:]

Important to point out the location of cranial nerve 8 which is on enters the internal
auditory meatus to transmit afferent signals to the brainstem and up.
[picture: vestibular schwannoma]
The most common cerebellopontine angle tumor is vestibular schwannoma. It is
usually called acoustic neuroma even if the origin of the tumor is in the verstibular nerve
sheath. Like other Cerebellopontine tumor, it causes slowly progressive unilateral
sensorineural hearing loss and vestibular hypofunction. However, these patients
typically do not have vertigo, because the gradual deficit is compensated centrally as it
develops.
Vestibular schwannoma typically starts at the internal auditory canal. Most
tumors grow slowly without causing symptoms for years, as may be in the case of our
patient who was apparently well before 6 months PTA. As the tumor enlarges, it
compresses the auditory nerve and causes sensorineural hearing loss with tinnitus as
manifested in the physical exam. Although the vestibular portion of CN VIII is involved
also, symptoms related to this portion of the nerve, such as vertigo and disequilibrium
are uncommon presenting symptoms because of the compensation of other organs for
balance which are the eyes and the cerebellum.
The growth rate is between 0.2 cm/year to as fast as 1 cm/year on imaging
studies
[picture 3]
As the tumor grows, it goes to the roomy posterior fossa until its size causes an
additional symptom.
[picture 4 and 5]
As the now significantly larger schwannoma expands to reach the nerves,
additional damage can occur with the compression of trigeminal nerve and facial nerve,
as manifested by numbness of the let face and shallow left nasolabial fold, respectively
[Kulang slide]
walang slide ito pero i think dapat meron---With a tumor size greater than 4 cm, the
cerebral aqueduct and fourth ventricle becomes gradually compressed and blocked
leading to impaired flow of the cerebrospinal fluid and subsequent development of a
noncommunicating or obstructive hydrocephalus, revealed in a cranial MRI in the case.

The expansion of ventricles proximal to these areas blocked by the tumor due to the
increased CSF volume elevates the intracranial pressure, which our patient complains
of as headache.
Side note: The large, lobulated cystic mass found on the MRI reveals that the tumor is of the
Antoni type B pattern of schwannoma. In this pattern, the tumor is less densely cellular and
consists of a loose meshwork of cells, microcysts, and myxoid stroma (Robbins). Mucinous and
cystic changes occurs, and when confluent, large cysts develop.

[chart]
With further growth, the cerebellum is compressed which causes decreased
compensation for vestibular deficit.
[Graph]
With cerebellar dysfunction and vestibular dysfunction, the visual system cannot
compensate adequately, and eventually causes imbalance and poor equilibrium as
manifested by difficulty in ambulation, unsteady gait, imbalance and dizziness after
sudden head movement.

C. Differential Diagnosis

1. Vestibular schwannoma
According to Adams,it occurs occasionally as part of von Recklinghausen
neurofibromatosis, in which case it takes one of two forms - NF1 or NF2. In the classic
von Recklinghausen neurofibromatosis, peripheral or NF type 1, schwannoma may
sporadically involve the eight nerve, usually in adult life. Rarely do bilateral vestibular
schwannoma occur in this type. This is opposite to NF type 2, which occurs bilaterally
before the age of 21 and show a strong (autosomal dominant) heredity.
Since there were no family members with the same illness, we presumed that
there might have been a sporadic mutation which could have been triggered by
environmental factors such as smoking, alcohol and/ or radiation or could have been
due to the age of the patient.
With the mutation of the tumor suppressor gene, Neurofibromin, in NF type 1,
there is persistently and uncontrolled activation of RAS leading to unregulated cellular

proliferation, clonal expansion and tumor progression. With the tumor progression,
adjacent structures can be affected by the mass effect.

While there are limited data as to the genetic pathology of unilateral vestibular
schwannoma, Robbins (and Cotrans) stated that even sporadic schwannomas are
commonly associated with inactivating mutations in the NF2 gene, a tumor supressor
gene (TSG), on chromosome 22. This gene produces a product (called merlin) which
normally restricts the cell-surface expression of growth factor receptors, such as EGFR;
with loss of function, cells hyperproliferate inresponse to growth factors thus leading.
Another genetic etiology is an NF1 of chromosome 17 loss of function, which can
develop in 5% of cases (Acoustic Neuroma Association of Canada). This gene produces
neurofibromin that inhibits RAS, which is an oncogene that switches on others genes
related to cell growth and division; with loss of function leads to persistently activated
RAS and an unregulated cellular proliferation leading to tumor progression.
[Next slide, please.]