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|LETTERS TO EDITOR
|Year : 2018 | Volume
| Issue : 6 | Page : 1800-1802
Adult post-varicella small vessel vaculopathy mimicking hypertensive basal ganglia haemorrhage with coexisting infarcts
Kamble J Harsha1, K Parameswaran2
1 Department of Neuroimaging and Endovascular Neurosurgery, Indo-American Hospital: Brain and Spine Centre, Vaikom, Kerala, India
2 Department of Neurology, Indo-American Hospital: Brain and Spine Centre, Vaikom, Kerala, India
|Date of Web Publication||28-Nov-2018|
Dr. Kamble J Harsha
Department of Neuroimaging and Endovascular Neurosurgery, Indo-American Hospital: Brain and Spine Centre, Chemmanakary - 686 143, Vaikom, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Harsha KJ, Parameswaran K. Adult post-varicella small vessel vaculopathy mimicking hypertensive basal ganglia haemorrhage with coexisting infarcts. Neurol India 2018;66:1800-2
|How to cite this URL:|
Harsha KJ, Parameswaran K. Adult post-varicella small vessel vaculopathy mimicking hypertensive basal ganglia haemorrhage with coexisting infarcts. Neurol India [serial online] 2018 [cited 2019 Jan 17];66:1800-2. Available from: http://www.neurologyindia.com/text.asp?2018/66/6/1800/246293
Virus-induced vasculopathies are an uncommon but increasingly recognized causative factors for the development of stroke and transient ischemic attack in children and adults. Varicella zoster, human immunodeficiency virus (HIV), and cytomegalovirus are the commonly associated viruses with stroke. While primary varicella infection is a common cause of neurological manifestations in children, secondary reactivation of varicella-zoster is common in immunocompromised adults. Both small and large vessels are involved, commonly resulting in cerebral ischemia or infarct. Rarely, subarachnoid hemorrhage, with or without an intraparenchymal component, is reported. We report a rare case of adult post-varicella vasculopathy involving small vessels resulting in a coexisting infarct as well as basal ganglia haemorrhage that mimicked the manifestations of a hypertensive bleed.
A 30-year-old male patient presented with a history of sudden onset of severe headache, left-sided weakness, slurring of speech, and facial deviation to the right side. He was taken to a local hospital and computed tomography (CT) scan was performed. The diagnosis of right basal ganglia bleed was established. His initial medical management was performed in the local hospital and then he was referred to our centre for further management. He and his younger sister, aged 25 years, had a history of chickenpox 1month prior to the ictus, which was uneventful.
His blood pressure was elevated at the time of ictus; however, when he came to our hospital, after 20 days of ictus, his blood pressure was normal. He had no past history of hypertension or diabetes mellitus. Both the patient and his sister were having the typical, multiple small-scars associated with hyperpigmention at sites corresponding to the previous varicella rash on the face and trunk. His neurological examination revealed left hemiparesis with left-sided mild facial palsy. He had significant improvement in the weakness from the itme of ictus until his presentation to our hospital. His blood investigations revealed normal total leucocyte count with mild neutrophilia and an erythrocyte sedimentation rate of 25 mm/h; his human immunodeficiency virus test was negative. His platelet count, prothrombin time and activated partial thromboplastin time were normal. His magnetic resonance imaging (MRI) showed a subacute intraparenchymal hematoma in the right external capsule-putaminal region [Figure 1] a, [Figure 1] b, [Figure 1] c, [Figure 1]d. An acute infarct was seen involving the right lentiform nucleus and caudate nucleus [Figures 1e andf]. Contrast MRI with a dedicated protocol to look for large-vessel wall enhancement was negative for vasculitis [Figure 1]g,[Figure 1]h,[Figure 1]i. Another acute lacunar infarct was seen in the right cerebral peduncle [Figure 1]j,[Figure 1]k,[Figure 1]l. Time-of-flight MR angiography revealed a mild diffuse attenuation of the right M1 segment of the middle cerebral artery, possibly due to mass effect from the hematoma or edema. Rest of the intracranial arteries were normal in calibre. Digital subtraction angiogram (DSA) was suggested; however, the patient was reluctant to undergo the invasive procedure due to his improving clinical condition. He was admitted and medically managed along with neurorehabiliatation. A short course of steroids and acyclovir injections were given with the presumptive diagnosis of small-vessel varicella vasculitis. He showed significant improvement in his symptoms, became ambulant over a period of 25 days. He showed further improvement in his neurological condition after a month's follow-up.
|Figure 1: (a-c) Axial T1W, T2 and FLAIR MR images showing subacute right external capsule-putamen hemorrhage (d) GRE image showing peripheral rim of blooming (e and f) DWI & ADC images showing right putamen and caudate nucleus acute infarct (g-i) Post contrast T1W images showing lack of vessel wall enhancement. (j-l) Axial FLAIR, DWI, ADC images showing lacunar infarct in the right cerebral peduncle|
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Varicella-zoster vasculopathies are caused by virus infections of the cerebral arteries either during primary infection (chicken pox) or during subsequent reactivation. The spectrum of vascular pathologies include ischemic infarctions, aneurysms, dissection, subarachnoid hemorrhage, or intraparenchymal hemorrhage. Both large and small arteries are commonly involved, and less often, pure small or large vessel disease is seen. Presence of large artery disease is diagnosed by the characteristic segmental constriction or post-stenotic dilatation on the DSA or MR/CT angiogram images. Demonstration of direct vessel wall enhancement by high quality post-contrast MRI can also help in the diagnosis. However, the direct demonstration of small vessel involvement is difficult because the angiogram is often negative. In such cases, the diagnosis is based on cerebral parenchymal changes, seen on MRI.
The common location for the infarction in varicella-zoster vasculopathy is the gray–white matter junction. The infarcts in the present case were in the right lentiform nucleus and head of the caudate nucleus in the lenticulostriate artery territory; a lacunar infarct was also seen in the right cerebral peduncle. Both the infarcts were suggestive of small vessel vasculopathy. Although previously described in literature, both these locations are uncommon sites for varicella small vessel vasculopathy. MR angiogram was negative for the presence of a large vessel vasculopathy. The temporal course of the initial varicella infection and appearance of neurological symptoms 1month later were in accordance with the typical natural course of varicella vasculopathy.
Subarachnoid haemorrhage (SAH) is more common in varicella-induced vasculitis than is intraparenchymal hemorrhage.,, The previously reported intraparenchymal hemorrhage was in the lobar location and was associated with SAH; however, the case was of secondary reactivation of varicella-zoster virus in adult life and not due to a primary varicella infection. The other case described by Danchaivijitr et al., was of a 7-month-old child and the location of the bleed was in the interhemispheric region and in the subarachnoid space without significant intraparenchymal component. In both the cases, the possible vessel involved was either a large artery or a small lobar artery. In the present study, the location of the bleed was in the right putamen-external capsular region, which was secondary to lenticulostriate artery involvement. The location of the bleed in our case was typical of that seen in a hypertensive bleed. Our case is the first one where an adult patient with a primary varicella infection developed an intraparenchymal bleed, coexistent with an infarct attributed to small vessel vasculopathy. The presumed mechanism of bleed appeared to be extensive vessel wall inflammation and an aggressive viral invasion of the tissues that was associated with necrotizing angiitis. It is possible that the same pathogenesis led to one small artery undergoing necrosis and causing a bleed, and the other small artery undergoing an occlusion causing an infarct.
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