Baylor University Medical Center Proceedings April 2017 - 219

the patient had some renal decompensation. It was thought that
her fever was neurogenic, and a second CSF sample 11 days
after the ﬁrst also did not exhibit any growths (with red blood
cell count of 7 K/μL; white blood cell count, 2 K/μL; proteins,
20 mg/dL; and glucose, 62 mg/dL).
The patient experienced steady clinical improvement prior
to discharge to inpatient rehabilitation after being in the hospital
for 17 days. After 2 weeks in rehabilitation, she developed a
drug rash to an unclear source with eosinophilic leukocytosis
and transaminitis leading to cessation of her phenytoin and
amoxicillin/clavulanic acid. She ultimately stayed in a longterm acute care facility and went home with minimal left-sided
weakness, slight diﬃculty with word ﬁnding, some visual ﬁeld
deﬁcits, and labile emotions.

DISCUSSION
RCVS is characterized by sudden onset of severe headaches
described as "thunderclap" with reversible, segmental, bilateral,
diﬀuse, and multifocal constriction of cerebral arteries (1-3).
There is a clear female predilection, with a mean age of onset
around 42 years (4). The headaches are recurrent in more than
95% of patients and occur over the course of 1 to 3 weeks (4, 5).
The headaches are described as bilateral, with posterior onset
and subsequent generalized pain. They can last for minutes to
Figure 2. (a) Focused three-dimensional rendering of the source data of the
intracranial CT angiogram (frontal view) shows severe right supraclinoid internal
days with an average of four attacks (4). A single attack is uncarotid artery stenosis (medial arrow) and mild right M1 segment middle cerebral
common (3). Moderate headaches are seen between the more
artery stenosis (lateral arrow). (b) Corresponding image from a digital subtracsevere bouts, accompanied by nausea, vomiting, photophobia,
tion angiogram obtained 4 days after the CT angiogram shows resolution of the
and phonophobia (6). In 8% to 40% of patients, RCVS can also
referenced sites of intracranial vascular stenoses.
present with transient or persistent neurologic deﬁcits, including
encephalopathy, dysarthria, ataxia, focal numbness, weakness,
seizures, and visual changes such as scotomas, blurry vision,
18 mg/dL; and glucose, 67 mg/dL. CSF cultures did not show
bacterial or fungal growth, and the IgG index was normal. The
hemianopsia, and cortical blindness (3, 4). Visual symptoms
patient was treated aggressively with lorazepam, fosphenytoin,
are the most commonly encountered. Seizures occur in up to
levetiracetam for seizures, and aspirin and verapamil for strokes.
17% of RCVS cases (3).
Conventional intracranial catheter angiography performed
Digital subtraction cerebral angiography reveals no abnor4 days after computed tomography angiography revealed commalities within the ﬁrst week of symptom onset in 30% of
plete resolution of the stenoses. Initial empiric antibiotics with
cases (4). Subsequently, a "string and beads" or "sausage on a
ceftriaxone and vancomycin were stopped at 5 days since CSF,
string" appearance develops that corresponds to generalized
urine, and blood cultures returned without any growths and
bilateral and diﬀuse segmental constriction and dilation of cerebral arteries (3, 5). One-third
of patients can develop ischemic or hemorrhagic strokes
(1). RCVS typically resolves
spontaneously within 3
months.
Diagnostic criteria for
RCVS have been proposed by
Calabrese et al (7) and include
1) multifocal segmental cerebral artery vasoconstriction
on direct or indirect cerebral
angiography; 2) no evidence
of aneurysmal subarachnoid
hemorrhage; 3) normal or
near-normal CSF analysis
(normal glucose, protein
Figure 3. Electroencephalogram recording shows left brain onset partial seizure (15 sec/page, 10 mm/sec, 7 uV/mm).
April 2017

Right hemispheric reversible cerebral vasoconstriction syndrome in a patient with left hemispheric partial seizures

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Table of Contents for the Digital Edition of Baylor University Medical Center Proceedings April 2017