1. 02/03/2013
CLINICAL PRESENTATION A 41 year old man with sudden onset
headache and diplopia.
Background:
Dr. Juan Carlos Díaz Torre A 41-year-old man presents to the ED with an acute
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severe headache, associated diplopia, photophobia,
Pediatra Neonatólogo nausea, vomiting and fever. The patient also has mild
dr_diaz_torre@hotmail.com gynecomastia.
(779) 100 . 40 . 26 HINT: The neurologic findings on this patient's physical
examination aid in establishing the etiology of his
1 headache. 2
He denies experiencing any associated seizures,
A 41-year-old man presents to the emergency focal weaknesses, previous similar episodes,
department (ED) complaining of a severe frontal frequent headaches, or previous visual disturbances.
headache that began suddenly and awakened him from
sleep. The headache is associated with nausea, He does not have any prior significant medical
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vomiting, and subjective fevers. problems, and his only medication is occasional
sildenafil.
He also complains of new-onset diplopia and
photophobia, but denies any decrease in visual acuity. He drinks socially, does not smoke, and denies
recreational drug use.
3 4
On physical examination, the patient is ill-appearing
but alert and in no apparent distress. His vital signs
reveal a temperature of 103.1°F (39.5°C), a blood
pressure of 155/95 mm Hg, and a pulse of 110 bpm.
The ocular examination demonstrates ptosis of the right
eye (see Figure 1), which is deviated inferolaterally and
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has a dilated and unreactive pupil (see Figure 2).
The visual field examination demonstrates bitemporal
hemianopsia. Funduscopic examination shows normal
venous pulsation and mild bilateral temporal disc pallor.
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2. 02/03/2013
Laboratory investigations reveal a hemoglobin
The cranial nerves are otherwise without deficit. The concentration of 13 g/dL (130 g/L); a white blood cell
neck is supple and without meningismus. Examination (WBC) count of 16.0 × 103/µL (16.0 × 109/L), with 75%
of the chest reveals mild bilateral gynecomastia, without neutrophils; and a platelet count of 340 × 103/µL (340 ×
nipple discharge. The lungs are clear to auscultation. 109/L). The electrolyte, blood urea nitrogen (BUN),
Cardiac auscultation reveals a normal S1 and S2 and no creatinine, and glucose examinations are all within
murmurs, rubs, or gallops.
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normal limits.
The abdomen is soft and nontender, and no Cerebrospinal fluid (CSF) specimens show 420,000 red
organomegaly is detected. Bilateral upper and lower blood ceels (RBC)/μL, 20,000 WBC/μL, a normal glucose
extremity strength is 5/5, with normal deep tendon and of 85 mg/dL (4.72 mmol/L), and an elevated protein
plantar reflexes. The patient's sensation is intact to light concentration of 230 mg/dL (2.3 g/L). The CSF Gram
touch and pinprick throughout, and the gait is normal. 7 stain is negative for bacteria. 8
A computed tomography (CT) scan of the What is the most likely diagnosis?
brain is performed, followed immediately by
magnetic resonance imaging (MRI); (see - Subarachnoid hemorrhage
Figure 3).
- Cerebellar infarction
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- Pituitary tumor apoplexy
- Cavernous sinus thrombosis
9 10
Discussion
Your Colleagues Responded:
The noncontrast CT scan of the brain showed a 2-cm
- Subarachnoid hemorrhage 13% sellar mass, with suprasellar extension. There was
impingement on the optic chiasm and the
- Cerebellar infarction 3% hypothalamus, with upward displacement. There was
increased density on the right side of the mass, which
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- Pituitary tumor apoplexy **** 61% was suggestive of hemorrhage.
- Cavernous sinus thrombosis 22% The sagittal and coronal T1 and T2 MRI scans
demonstrated a large soft-tissue mass in the pituitary
fossa, with areas of intermediate- and high-intensity
11 signal suggestive of hemorrhage (see Figure 3). 12
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3. 02/03/2013
Coronal gadolinium-enhanced T1 images
revealed that the mass had a heterogeneous These findings are consistent with pituitary
pattern of faint peripheral enhancement apoplexy as a result of hemorrhage with or
(Figure 4). without infarction, likely into a pituitary adenoma.
There was evidence of mass effect on the right Tests for evaluating the hormonal status of the
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cavernous sinus, which was most evident in patient revealed panhypopituitarism. Prior to the
the coronal T1- and T2 images. acute apoplectic episode, the patient had findings
suggestive of central hypogonadism, probably as a
component of his hypopituitarism caused by
pituitary macroadenoma (diminished libido and
13
bilateral gynecomastia). 14
His neurologic finding (right-sided ptosis with a
fixed and dilated pupil pointing downward and
outward) was consistent with a right-sided 3rd The word "apoplexy" stems from a Greek term
nerve palsy caused by extension of hemorrhage meaning to "have a stroke".
into the right cavernous sinus.
Neurologic symptoms and signs are secondary to
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Pituitary tumor apoplexy is defined as displacement of the optic nerve and impingement
hemorrhage or infarction of a pituitary gland of the 3rd, 4th, and 6th cranial nerves.
associated with the presence of a preexisting
pituitary adenoma. It manifests as a sudden, Hormonal dysfunction results from destruction of
severe headache, and it is sometimes associated the anterior pituitary gland.
with neurologic and hormonal dysfunction.
15 16
Pituitary tumor apoplexy is a rare disorder with an Pituitary tumor apoplexy is only rarely associated
annual incidence of about 1.2 per million. Men are with a healthy gland; however, approximately 50%
affected twice as often as women, and all age of patients who present with pituitary tumor
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groups can be affected, with the majority of apoplexy are not diagnosed with a pituitary lesion
patients in the 5th or 6th decades of life. It is prior to their presentation. All types of pituitary
estimated to occur in 1.5-27.7% of cases of tumors carry the same risk for apoplexy.
pituitary adenoma.
17 18
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4. 02/03/2013
The most common symptom of pituitary tumor The increase in intrasellar pressure results in many
apoplexy is headache. Almost all patients of the symptoms and signs of pituitary tumor
describe a sudden, severe retro-orbital or apoplexy.
bifrontal headache, which is associated with
vomiting in two-thirds of cases. Laterally, the increased pressure causes
compression of the structures in the cavernous
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The headache and vomiting result from the sinus, namely the 3rd, 4th, and 6th cranial nerves,
sudden increase in intrasellar pressure either with the 3rd being most commonly affected as a
caused by the hemorrhage or secondary to result of its vulnerable position (parallel to the
meningeal irritation from blood or tumor lateral wall of the pituitary gland). The 6th cranial
products that leak into the CSF. nerve is the least commonly involved because of its
most lateral location within the sinus.
19 20
Ophthalmoplegia (caused by 3rd, 4th, and 6th
nerve palsies or any combination thereof) is
present in around 80% of patients presenting with Blood leaking into the subarachnoid space may
pituitary tumor apoplexy. result in chemical meningitis with fever,
meningismus, and photophobia. Fever in patients
Also located within the cavernous sinus is the with apoplexy may also be explained by alteration in
trigeminal nerve; its involvement may cause facial thermal regulation caused by hypothalamic
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pain or sensory loss. Carotid siphon compression involvement by the hemorrhage or by adrenal
may present as hemiplegia. Superiorly, the insufficiency associated with hypopituitarism.
increased pressure compresses the optic chiasm,
optic tract, or optic nerve, leading to decreased Hemorrhage may extend into the brain parenchyma
visual acuity or visual field defects (classically, causing cortical irritation and provoking seizures.
bitemporal hemianopsia). 21 22
The elevated intrasellar pressure also accounts for
the endocrine abnormalities found in cases of Although not common, patients with pituitary tumor
pituitary tumor apoplexy. This pressure increase apoplexy may have diabetes insipidus at presentation.
results in compression of the pituitary tissue, The true etiology of diabetes insipidus in this setting
compromising its vascular supply and leading to is unknown, but it may result from the increased
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hypopituitarism. pressure on the pituitary infundibulum, which
impedes the antidiuretic hormone from passing from
Adrenal insufficiency is the most clinically significant the hypothalamus to the posterior lobe of the
result of hypopituitarism, contributing significantly pituitary.
to the mortality of patients with pituitary tumor
apoplexy if not promptly recognized and treated.
23 24
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5. 02/03/2013
Pituitary tumor apoplexy following childbirth
A precipitating factor is identified in 50% of cases of associated with significant postpartum hemorrhage
pituitary tumor apoplexy. Predisposing factors include in nontumorous glands is termed "Sheehan
dopamine agonist treatment, head trauma, pituitary syndrome".
irradiation, pregnancy, coronary artery bypass
grafting, surgical operations, and anticoagulation. The hypertrophy of the pituitary gland that occurs in
normal pregnancy combined with the arterial spasm
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Endocrine stimulation tests are also associated with of the pituitary's blood supply (caused by bleeding
pituitary tumor apoplexy. It is postulated that and hypotension) both contribute to the
hormones used in these tests may increase blood flow development of Sheehan syndrome; however the
in pituitary adenomas, provoking bleeding in friable clinical presentation of pituitary apoplexy in these
vessels. cases is usually less dramatic, with a more gradual
25 development of signs and symptoms of 26
hypopituitarism.
Once recognized, effective treatment of pituitary
tumor apoplexy requires prompt administration of
The diagnosis of pituitary tumor apoplexy is best high-dose corticosteroids. Steroids should be
established by MRI; however, this is usually preceded administered in supraphysiologic doses to not only
by a rapid diagnostic CT scan to screen for intracranial replace endogenous hormone deficiency during a
hemorrhage. MRI is superior to CT scanning for stressful condition, but also to take advantage of its
evaluating the pituitary gland and possibly visualizing
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anti-inflammatory effect by decreasing swelling on
hemorrhage not seen by CT. parasellar structures.
In one study, the detection rate of pituitary tumor The definitive treatment for pituitary tumor
apoplexy by CT scanning was 21%, whereas the apoplexy is emergent surgical decompression.
detection rate was 100% with MRI. Transsphenoidal resection is the most common
27 approach in this situation. 28
In cases where there is significant extension of With prompt recognition, timely surgery, and proper
hemorrhage into the brain parenchyma beyond the medical management, the majority of patients with
diaphragma sella, an intracranial approach may be pituitary tumor apoplexy improve. Ophthalmoplegia
preferred. In a minority of cases, conservative medical is usually the first symptom to resolve. Less readily
therapy is an acceptable alternative; examples of this restored is the optic nerve defect resulting in
include patients who are poor surgical candidates and decreased visual acuity and restricted visual fields.
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selected patients who present with isolated
meningismus or ophthalmoplegia and show More than half of patients, however, will have
significant improvement with steroid administration. permanent hormone deficiencies resulting from
pituitary injury and will require hormone
Medical management includes monitoring of replacement.
endocrine, neurologic, and ophthalmologic function 29 30
combined with hormone replacement.
.
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6. 02/03/2013
One study showed that maintenance steroid,
thyroid hormone, and testosterone replacement An endocrinology evaluation was completed, and
was essential postoperatively in 82%, 89%, and 64% the patient was confirmed to have hypopituitarism.
of patients, respectively. Two weeks following surgery, his ophthalmoplegia
and visual field deficits had completely resolved.
Following immediate administration of high dose
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corticosteroids, the patient in this case underwent Pharmacologic management of the patient's
an emergent transsphenoidal resection. An infarcted hypopituitarism included replacement therapy with
adenoma was identified, with extensive areas of corticosteroids, levothyroxine, and testosterone.
hemorrhage and necrosis consistent with apoplexy.
Resolution of the headache and improvement of
visual and extraocular function were noted 24 hours 31 32
after surgery.
A patient presents to the ED with a rapid onset of
headache accompanied by diplopia, photophobia,
and decreased visual acuity, raising concern for a
number of potentially serious conditions. Which of
Your colleagues responded:
the following diagnostic tests is most likely to assist
in confirming pituitary tumor apoplexy as the
- Lumbar puncture 1%
diagnosis?
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- MRI of the brain **** 89 %
- CT of the brain 10 %
- Lumbar puncture
- Carotid ultrasonography 0%
- MRI of the brain
- Dilated retinal examination 0%
- CT of the brain
- Carotid ultrasonography
- Dilated retinal examination 33 34
The patient you are examining is confirmed to have
pituitary tumor apoplexy. Which of the following
The diagnosis of pituitary tumor apoplexy is best
choices, if they were part of this patient's history, is a
established by MRI. Although this may be
potentially responsible precipitating factor for this
preceded by a CT scan to screen for intracranial
occurrence?
hemorrhage.
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- Coronary artery bypass graft
Lumbar puncture may reveal xanthochromia
- Head trauma
and/or red blood cells, but this may also be seen in
- Endocrine stimulation tests
subarachnoid hemorrhage resulting from a
- Dopamine agonist treatment
ruptured aneurysm.
- All of the above
35 36
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7. 02/03/2013
Your colleagues responded:
- Coronary artery bypass graft 1%
- Head trauma 5% Gracias por su atención
- Endocrine stimulation tests 7%
- Dopamine agonist treatment 4%
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- All of the above **** 83 % Dr. Juan Carlos Díaz Torre
Pediatra Neonatólogo
Predisposing factors for pituitary apoplexy include
bromocriptine treatment, head trauma, pituitary dr_diaz_torre@hotmail.com
irradiation, pregnancy, coronary artery bypass (779) 100 . 40 . 26
grafting, surgical operations, anticoagulation, and 37 38
endocrine stimulation tests.
7