Atypical Migraine turns out to be pseudotumor cerebri

Introduction
Headache is the most frequent symptom of Pseudotumor cerebri syndrome (PTCS), but
there is considerable overlap between the headache features of raised intracranial
pressure and the headache features of primary headache disorders. Patients with
pseudotumor cerebri frequently have other types of headaches, not necessarily related
to increased intracranial pressure, and hence it would be difficult to diagnoses the
patient on initial presentation with atypical presentation. Sixty-eight percent of patients
with pseudotumor cerebri had definable headache disorders, including episodic tension
type headache (30%) and migraine without aura (20%) (1). We report a case of
diagnostic dilemma, a case of 24 Year old female with history of Migraine disorder
presented with atypical headache.
Case description
24 year-old Female presented with sudden severe headache associated with decreased
sensation and tingling of left upper and lower extremities started suddenly. Her
headache was localized on right frontal and occipital head and neck, aggravated with
bending head, associated with blurry vision, dizziness, flashing lights, black spots and
anosmia. Black spot's, location of the headache and decreased sensation were new
compared to her usual migraines. Her past medical history was significant for Migraine
diagnosed at age of 13 and recurrent episodes of Bells palsy. She was not on any
medications including oral contraceptive. Review of system was negative for fever,
chills, nausea, vomiting, seizure, loss of consciousness, photophobia, dizziness and
rash. Positive neurological physical examination findings included: decreased
sensations in left trigeminal nerve (V1-V2), decreased sensation in left upper and lower
extremities. Ophthalmologic evaluation was negative for papilledema. Magnetic
resonance imaging (MRI) of the brain and MR angiography of head and neck were
normal. She was initially managed as case of severe migraine headache..,
however showed minimal improvement. On further examination revealed worsening
headache with cough and valsalva. Thus, Suspicious was made for pseudotumor
cerebri and Lumbar puncture was performed, consistent with opening pressure of 39
cmH20. 20 CC of Cerebrospinal fluid was drained and her significant improvement in
symptoms was seen.

Discussion
We report a case of young women who had history of migraine headache since age of
13, presented with headache and associated sensory aura and later diagnosed as case
of pseudotumor cerebri without papilledema. Pseudotumor cerebri in adults is almost
exclusively a disease of overweight women of childbearing age. Headache is most
common presenting symptom and found in 75-94% of the patient(2). Headache is
described as pressure-like, holocranial, frontal or retro-orbital, worse on waking or with
Valsalva-type manoeuvres, and improve with CSF removal(2). Other associated

symptoms commonly found are nausea with or without vomiting (72-75%), photophobia
or phonophobia or both (42-73%) , pulsatile tinnitus(52-60%), back pain (52%), neck
pain(42%), visual loss(32%), radicular pain(19%) and diplopia (18%)(2). Papilledema is
considered as hallmark of pseudotumor cerebri and important physical finding present
in patient with pseudotumor cerebri. The diagnosis of pseudotumor cerebri become
difficult when papilledema is not present. Atypical case presentation of pseudotumor
cerebri are well described in adult literature in which papilledema is found to have
absent in (5-14 %) in studies(3). Diagnosis is often delayed when papilledema is
absent. Early diagnosis and intervention is required to manage headache and prevent
visual morbidities. Headache worsened by coughing commonly associated with
increase in intracranial pressure and could be positive finding in pseudotumor cerebri ,
other intracranial hypertension disorders, post-ictal headache, migraine (53-87%) and
tension headache (29%) (4). Thus very important physical finding if performed to
diagnose case of pseudotumor cerebri without papilledema.
Conclusion
Its difficult to diagnose a case of pseudotumor cerebri without papilledema as it shares
similar symptoms with migraine and other headache disorders and around 68% patient
with pseudotumor cerebri found to have classified headache disorders, and thus
precipitation of headache with Valsalva maneuvers (ie, coughing, sneezing, bearing
down) in absence of any structural abnormalities on MRI should raise suspicion for
pseudotumor cerebri and lumbar puncture should be done.

References
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intracranial hypertension. Neurology. 2002;58(10):1551-3.
2. Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic
intracranial hypertension: mechanisms, management, and future directions.
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3. Digre KB, Nakamoto BK, Warner JEA, Langeberg WJ, Baggaley SK, Katz BJ. A
Comparison of Idiopathic Intracranial Hypertension With and Without
Papilledema. Headache. 2009;49(2):185-193.
4. Cordenier A, De hertogh W, De keyser J, Versijpt J. Headache associated with
cough: a review. J Headache Pain. 2013;14:42.