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|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 1 | Page : 91-92
An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period
Amita Bhargava1, Basavaraj F Banakar1, Guruprasad S Pujar1, K Shubhkaran1, Hemant Jangid2
1 Department of Neurology, Dr. S. N. Medical College, Jodhpur, Rajasthan, India
2 Satyam MRI Centre, Dr. S. N. Medical College, Jodhpur, Rajasthan, India
|Date of Submission||04-Jan-2014|
|Date of Decision||06-Jan-2014|
|Date of Acceptance||30-Jan-2014|
|Date of Web Publication||7-Mar-2014|
Basavaraj F Banakar
Department of Neurology, Dr. S. N. Medical College, Jodhpur, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bhargava A, Banakar BF, Pujar GS, Shubhkaran K, Jangid H. An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period. Neurol India 2014;62:91-2
|How to cite this URL:|
Bhargava A, Banakar BF, Pujar GS, Shubhkaran K, Jangid H. An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period. Neurol India [serial online] 2014 [cited 2019 Sep 17];62:91-2. Available from: http://www.neurologyindia.com/text.asp?2014/62/1/91/128349
A 29-year-old primigravida of 34 weeks amenorrhea was referred with the complaints of severe episodic headaches, vomiting and status epilepticus after emergency cesarean section from gynecology services for evaluation. History was negative for fever, hypertension, trauma, pedal edema, abortions/fetal loss or epilepsy and she had regular antenatal care. On examination, she was stuporous with left hemiparesis and bilateral plantar extensor. Magnetic resonance imaging (MRI) brain revealed multiple large acute intrparenchymal hemorrhages: Right frontal lobe (4 × 3.8 × 2.9 cm), left superior frontal lobe (1.2 × 1 cm) and left temporo-occipital lobe (1.4 × 2 cm) with intraventricular extension [Figure 1]a and b. Fluid-attenuated inversion recovery sequence also revealed subarachnoid hemorrhage (SAH) [Figure 1]c. MR-angiography (MRA) revealed marked diffuse narrowing of supraclinoid segments of bilateral internal carotid arteries, proximal bilateral M1 segment of middle cerebral artery, A1 segment of anterior cerebral artery and P1 segment of posterior communicating artery [Figure 1]d with no evidence of cerebral aneurysm. MR-venogram (MRV) was normal. With a working diagnosis of reversible cerebral vasoconstriction syndromes (RCVS) she was treated with antiepileptic drugs, nemodepine and supportive therapy. Patient improved over the next 15 days and had minimal deficits when discharged. At 2 months follow-up repeat MRA was normal [Figure 1]e and patient was asymptomatic.
|Figure 1: Magnetic resonance imaging brain fl uid-attenuated inversion recovery (FLAIR) sequence showing acute parenchymal hemorrhage in right frontal lobe (a) left temporo-occipital lobe (b) diffuse FLAIR hyperintensity of bihemispheric sulcal spaces suggestive of subarachnoid hemorrhage (c) Magnetic resonance-angiography of brain showing marked diffuse narrowing of supraclinoid segments of bilateral ICA, M1, A1 and P1 segments (d) and showing normal vessels after 2 months (e)|
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In our patient with the clinical scenario, the differentials considered included: Eclampsia, cortical venous thrombosis (CVT), posterior reversible encephalopathy syndrome (PRES) and arterial stroke. Eclampsia was excluded as there was no history of hypertension and no albuminuria. MRI brain and MRV excluded the diagnostic possibilities of CVT and PRES. Cerebral MRA revealed bilateral multisegmental vasoconstriction, possibility of primary angiitis of central nervous system and RCVS were considered. Reversal of segmental vasoconstriction on repeat MRA confirmed the diagnosis of RCVS.
RCVS comprise a group of disorders characterized by prolonged but reversible vasoconstriction of the cerebral arteries, usually associated with acute-onset, severe, recurrent headaches, with or without additional neurologic signs and symptoms.  Description of this rare entity dates back to 1960s  and was limited to case reports. Call et al. reviewed the literature and published a case series titled 'Reversible Cerebral Segmental Vasoconstriction' in 1988.  Calabrese et al.  brought the awareness of this entity by their reviews. It is usually a disease of young and middle aged females. The exact incidence is unknown. Approximately 60% of cases are secondary, mainly postpartum period, eclampsia, exposure to vasoactive substances  such as cannabis, cocaine, immunosuppressants, sympathomimetics, methergine, bromocriptine etc. Although the pathophysiology remains poorly understood, current consensus appears to be alteration of cerebral vascular tone secondary to interaction between sympathetic overactivity and endothelial dysfunction, resulting in dysautoregulation.  Endothelin, endothelium-derived peptide, has been considered to participate in the vasospasm of preeclampsia.  Factors implicated in antepartum cases without pre-eclampsia/eclampsia are not yet clearly defined.
The major complications  are nonaneurysmal SAH, PRES, intracerebral hemorrhage, transient ischemic attacks and cerebral infarction. Til now, there are no specific guidelines for treatment, it is mainly based on expert opinion and case reports. The precipitating factors must be tackled aggressively and offending drugs if any, should be discontinued. Depending on the severity of vasospasm drugs such as nimodipine, verapamil, magnesium sulfate and milrinone can be tried.  RCVS often has a good prognosis.
Our patient was unusual in that RCVS occurring in antepartum period in a normal pregnancy without any precipitating factors. Is it just a co-occurrence or antepartum period can also be a risk factor is not certain, hence further study of large series of cases is warranted. RCVS though benign can be fatal at times. It can occur during antepartum period also. Clinicians should be aware of such a rare association so that early intervention will reduce the mortality and morbidity.
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