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We report a right-handed 19-year-old girl who developed reversible cerebral vasoconstriction syndrome (RCVS) lateralized to the right hemisphere with simultaneous new-onset left hemispheric seizures. RCVS, typically more diffuse, was lateralized to one of the cerebral hemispheres.
Reversible cerebral vasoconstriction syndrome (RCVS) is a self-limited, underrecognized clinicoradiologic syndrome characterized by recurrent thunderclap headaches and reversible, bilateral, and multifocal vasoconstriction of cerebral arteries. Calabrese et al proposed the name of this syndrome in 2007. We report a patient who had lateralized RCVS possibly due to the vasodilatory effects of seizures that were occurring on the contralateral side.
A right-handed 19-year-old girl with severe developmental disability, who could converse, make lunch, and ambulate, presented with nausea, vomiting, confusion, word-finding difficulty, and fever (100.5°F); when found by her mother, she was in the fetal position in pain. She had not had significant headaches in the past. The patient then developed left-sided hemiplegia over the course of 7 days. This was followed by recurrent right arm extension with some facial twitching, later confirmed to be frequent seizures by prolonged electroencephalogram. Her last known seizures occurred at 6 months of age and were attributed to vaccinations. Her blood pressure was not elevated, with an initial blood pressure of 102/68 mm Hg.
Magnetic resonance imaging of the brain revealed scattered foci of diffusion restriction throughout the right hemisphere indicating multifocal small infarctions and right hemispheric cortical edema (Figure 1). Magnetic resonance angiography and subsequent computed tomography angiography revealed severe right supraclinoid internal carotid artery narrowing and mild right M1-middle cerebral artery narrowing (Figure 2). No stenosis was present within the left intracranial arterial vasculature. Transcranial Doppler showed elevated velocities only in the right middle cerebral artery territory. An electroencephalogram showed right brain slowing and left brain interictal and ictal rhythmic sharps with partial seizures (Figure 3).
Cerebrospinal fluid (CSF) collected prior to antibiotic initiation was negative for infections: the red blood cell count was 43 K/μL; white blood cell count, 9 K/μL; protein count, 18 mg/dL; and glucose, 67 mg/dL. CSF cultures did not show bacterial or fungal growth, and the IgG index was normal. The patient was treated aggressively with lorazepam, fosphenytoin, levetiracetam for seizures, and aspirin and verapamil for strokes. Conventional intracranial catheter angiography performed 4 days after computed tomography angiography revealed complete resolution of the stenoses. Initial empiric antibiotics with ceftriaxone and vancomycin were stopped at 5 days since CSF, urine, and blood cultures returned without any growths and 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 long-term acute care facility and went home with minimal left-sided weakness, slight difficulty with word finding, some visual field deficits, and labile emotions.
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 days with an average of four attacks (4). A single attack is uncommon (3). Moderate headaches are seen between the more severe bouts, accompanied by nausea, vomiting, photophobia, and phonophobia (6). In 8% to 40% of patients, RCVS can also present with transient or persistent neurologic deficits, including encephalopathy, dysarthria, ataxia, focal numbness, weakness, seizures, and visual changes such as scotomas, blurry vision, hemianopsia, and cortical blindness (3, 4). Visual symptoms are the most commonly encountered. Seizures occur in up to 17% of RCVS cases (3).
Digital subtraction cerebral angiography reveals no abnormalities within the first week of symptom onset in 30% of cases (4). Subsequently, a “string and beads” or “sausage on a string” appearance develops that corresponds to generalized 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 <100 mg/dL, <15,000 white blood cells/μL); 4) severe, acute headaches, with or without additional neurologic signs or symptoms; and 5) reversibility of angiographic abnormalities within 12 weeks of onset.
RCVS may be primary (idiopathic) or secondary (60% to 80%) with multiple precipitants (5). The most common are vasoactive substance use and a postpartum state. The exact pathogenesis of RCVS is unknown, with “disturbance of cerebral vascular tone” being the most widely accepted hypothesis.
Primary angiitis of the central nervous system (PACNS) was recognized to be distinct from RCVS only in 1990 (3). Headaches tend to be more insidious in PACNS, which follows a more progressive-chronic course with a stepwise deterioration (3, 5). CSF is significant for a marked inflammatory profile and can also reveal oligoclonal bands. Brain magnetic resonance imaging may reveal deep infarcts and contrast enhancements of the vessel wall (3, 4). It is of some importance to accurately distinguish these two entities, since treatment for PACNS with corticosteroids can be harmful in RCVS. Conversely, RCVS stabilizes and improves more quickly (3). There is no specific proven therapy for RCVS.