A Case of Reversible Cerebral Vasoconstriction Syndrome: Postpartum Cerebral Angiopathy in a 32-Year-Old Primigravid Treated with Intravenous Immunoglobulin

This is a case of a 32-year-old primigravid who developed sudden severe headache on the 7th day postpartum associated with focal neurologic deficits and altered sensorium. She had a GCS score of 6, anisocoric pupils and an NIHSS score of 31. Cranial MRI with MRA showed multifocal hyperacute to acute infarcts on the left occipital lobe, left thalamus, and midbrain which was more prominent on the right. Due to clinical deterioration, a repeat Cranial MRI with MRA was done and showed progression of infarcts involving both thalami and right pons with interval appearance of contour irregularities in the proximal anterior cerebral, posterior cerebral, basilar and internal carotid arteries. Serial transcranial Doppler showed significant distal right middle cerebral artery vasospasm. She was managed as a case of reversible cerebral vasoconstriction syndrome, associated with postpartum cerebral angiopathy. Intravenous pulse methylprednisolone was started subsequently IVIG was initiated. Intravenous immunoglobulin was given for 5 days. The patient gradually improved, underwent rehabilitation therapy, and was discharged stable after 6 weeks.

Difficulties in the differential diagnosis of multiple sclerosis and Susak syndrome

Retino-cochleo-cerebral angiopathy or Susac syndrome is a rare autoimmune disease that selectively affects the vessels of the retina, the inner ear and the central nervous system. Differentiation of Susac syndrome and multiple sclerosis presents difficulties due to the similarity of MRI semiotics of these two diseases. This article presents two clinical cases of patients with Susac syndrome who were diagnosed with multiple sclerosis at the onset of the disease. Based on the analysis of our own clinical observations and literature data, the issues of differential diagnosis of Susac syndrome and multiple sclerosis are highlighted. For the first time a variant of the MRI picture transformation in Susac syndrome is presented.

A case of moyamoya disease in a patient with multi-drug resistant tuberculosis

Moyamoya cerebral angiopathy is characterized by progressive stenosis or occlusion of the internal carotid artery or its branches with subsequent development of basilar collaterals. It is commonly seen in Asian population. Authors present a case report of a 12 year-old boy with Multidrug resistant tuberculosis with Moyamoya disease. Moyamoya disease rarely coexists with tuberculosis. However, we can infer that tuberculosis may coexist in a patient in a patient of Moyamoya disease.

Microduplication of 15q13.3 and Microdeletion of 18q21.32 in a Patient with Moyamoya Syndrome

Moyamoya angiopathy (MA) is a cerebrovascular disease determining a progressive stenosis of the terminal part of the internal carotid arteries (ICAs) and their proximal branches and the compensatory development of abnormal “moyamoya” vessels. MA occurs as an isolated cerebral angiopathy (so-called moyamoya disease) or in association with various conditions (moyamoya syndromes) including several heritable conditions such as Down syndrome, neurofibromatosis type 1 and other genomic defects. Although the mechanism that links MA to these genetic syndromes is still unclear, it is believed that the involved genes may contribute to the disease susceptibility. Herein, we describe the case of a 43 years old woman with bilateral MA and peculiar facial characteristics, having a 484-kb microduplication of the chromosomal region 15q13.3 and a previously unreported 786 kb microdeletion in 18q21.32. This patient may have a newly-recognized genetic syndrome associated with MA. Although the relationship between these genetic variants and MA is unclear, our report would contribute to widening the genetic scenario of MA, in which not only genic mutation, but also genome unbalances are possible candidate susceptibility factors.

Curious case of corpus callosal hematoma in a normotensive post-partum female patient: a case report of reversible post-partum angiopathy with clinico-radiological correlation

Postpartum cerebral angiopathy (PCA) is a rare cause of stroke in the puerperium. It usually presents with episodes of headache, seizures and neurological deficits in early post-partum period in normotensive females. CT, MRI and catheter angiography may demonstrate segmental vasoconstriction that often resolves spontaneously. PPA is generally regarded as a benign, non-relapsing and reversible disease process. We present a clinico-radiological correlative case of isolated corpus callosal hematoma in a post-partum normotensive female with evidence of intracranial vascular spasm and luminal irregularity on CT and catheter angiography. Follow up MR angiography showed resolution of the vasospasm and luminal irregularity. Patient was managed conservatively and showed resolution of the symptoms in follow up visit on 8th week.

Cerebral Microhemorrhages and Meningeal Siderosis in Infective Endocarditis

Objective: Patients with infective endocarditis (IE) frequently experience cerebral insults, and neurological involvement in IE has been reported to herald a worse prognosis. In this manuscript, we describe a distinctive pattern of findings on susceptibility-weighted imaging (SWI) sequences in subjects with IE. Methods: Patients with IE who underwent SWI MRI at an academic hospital from 2009 to 2014 were retrospectively analyzed. The pattern of findings was compared to SWI findings in groups of subjects with cerebral amyloid angiopathy (CAA) or severe hypertension. Results: Sixty-six subjects with IE were included; 64 (94%) had microhemorrhages and the average number per patient was 21.5. In 11 (17%) patients, microhemorrhages were the only neuroimaging abnormality. The majority of microhemorrhages were between 1 and 3 mm. In a direct comparison of gradient-echo T2* (GRE-T2*) and SWI, many microhemorrhages in this size range were not detected by GRE-T2*. Microhemorrhages in IE involved every part of the brain with a significant predilection for the cerebellum. This pattern was distinct from that seen in hypertension or CAA. Small subarachnoid hemorrhage or meningeal siderosis were also frequently detected in IE, but were not associated with mycotic aneurysms. Interpretation: SWI is a sensitive diagnostic technique for detecting infectious cerebral angiopathy in subjects with IE, producing a pattern of microhemorrhages that were distinct from other common microangiopathies.