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Thunderclap headache and hemiparesis as presenting manifestation of adult moyamoya disease

1 Department of Neuromedicine, Bangur Institute of Neurosciences, Institute of Postgraduate Medical Education and Research and SSKM Hospital, Kolkata, West Bengal, India
2 Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
3 Department of General Medicine, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India
4 Department of Neurology, Krankenanstalt Rudolfstiftung, Messerli Institute, Vienna, Austria
5 Department of Neuro Medicine, R.G. Kar Medical College and Hospital, Kolkata, West Bengal, India
6 Department of Medicine, Kamineni Institute of Medical Sciences, Nalgonda, Telangana, India

Date of Submission24-Nov-2019
Date of Decision17-Jan-2020
Date of Acceptance11-Mar-2020

Correspondence Address:
Subhankar Chatterjee,
Department of General Medicine, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_320_19

How to cite this URL:
Dubey S, Ray BK, Ghosh R, Chatterjee S, Finsterer J, Lahiri D, Biswas R. Thunderclap headache and hemiparesis as presenting manifestation of adult moyamoya disease. Med J DY Patil Vidyapeeth [Epub ahead of print] [cited 2021 Jun 13]. Available from: https://www.mjdrdypv.org/preprintarticle.asp?id=309182

Dear Sir,

A 32-year-old HIV negative, euglycemic, euthyroid, nondyslipidemic, and normotensive female without a history of addiction or the use of oral contraceptives presented with sudden-onset first-ever holocranial thunderclap headache and left hemiparesis. The family history was negative for similar illness, headaches, focal sensorimotor or any cognitive deficit, seizures, visual symptoms, behavioral problems, and bladder-bowel dysfunction. On examination, vitals were normal. Neurological examination revealed a Glasgow Coma Scale score of E4V5M6, mild left hemiparesis (lower limb >upper limb) along with neck rigidity. Differential diagnoses considered were ruptured arteriovenous malformation (AVM) with intraventricular extension, ruptured aneurysm with subarachnoid hemorrhage (SAH), reversible cerebral vasoconstriction syndrome (RCVS), cerebral venous sinus thrombosis (CVST), central nervous system (CNS) vasculitis, primary intraventricular hemorrhage, dissection of large arteries of the neck with acute ischemic stroke, lobar hemorrhage with extension to meninges, bleeding inside an intracranial space-occupying lesion (ICSOL), neuroinfection, and pituitary apoplexy.

Cerebral computed tomography (CCT) demonstrated a large right frontal hemorrhage with extension to all ventricles without any other noteworthy abnormality [Figure 1]. The list of differentials was shortened to: (i) ruptured AVM; (ii) SAH- aneurysmal bleeding usually presents without significant parenchymal involvement, and it is quite unlikely that SAH presents with focal neurodeficits on the 1st day; (iii) CVST- ruled out for lack of papilledema, seizure, unilaterality of parasagittal lesion, and the absence of other signs of CVST on CCT; (iv) CNS vasculitis - unlikely as vasculitis usually does not initially present with macro-hemorrhage. Furthermore, the absence of long-standing headache and cranial nerve palsies rendered this possibility less likely; (v) RCVS known to manifest with multiple different arterial territory infarctions due to diffuse, profound vasospasm; (vi) dissection usually presents with infarct and not hemorrhage, and is associated with history of strenuous exercise, trauma, or neck manipulation; (vii) ICSOL bleeding- ruled out because it is unlikely to spill over to ventricles. They are usually small and multiple, usually not associated with headache; until intracranial tension increases.
Figure 1: Computerized tomography scan of the brain demonstrated a large right frontal hemorrhage with extension to all ventricles

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Surprisingly, digital subtraction angiography revealed bilaterally steno-occlusive lesions of the supraclinoid portion of the internal carotid arteries with multiple prominent, intra- and extracranial collaterals with classic “puff of smoke” appearance suggesting Moyamoya disease (MMD) [Figure 2] and [Figure 3]. Secondary causes of MMD were ruled out as workups for infectious processes, autoimmune profile, vasculitis profile, prothrombotic workup, hemoglobin electrophoresis, cerebrospinal fluid analysis, and karyotyping were negative. In adult MMD, hemorrhage is more common than in pediatric MMD due to the development of immature collaterals to overcome chronic cerebral hypoperfusion. In contrast, cerebral ischemic events are more common in children. Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults.[1] In MMD, headache is a frequently encountered symptom.[2] Causes of headache in MMD might be extensive collaterals, dilatation of extracranial vessels, neuroinflammation, intracerebral hemorrhage with intraventricular extension, which ultimately leads to subarachnoid extension resulting in headache.[3] However, this case is peculiar in the sense that it presented with “thunderclap” headache and hemorrhagic stroke without any commonly observed classical antecedent manifestations such as cognitive decline, seizures, headache, or neuropsychiatric abnormalities.
Figure 2: Digital subtraction angiography showing steno-occlusive lesions of the supraclinoid portion of both internal carotid arteries (right panel showing right internal carotid arteries and a left panel showing left internal carotid arteries)

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Figure 3: Digital subtraction angiography showing dense collaterals resulting in classical “puff of smoke” appearance

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Zipfel GJ, Fox DJ Jr., Rivet DJ. Moyamoya disease in adults: The role of cerebral revascularization. Skull Base 2005;15:27-41.  Back to cited text no. 1
Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK. Headache in pediatric moyamoya disease: Review of 204 consecutive cases. J Neurosurg 2005;103:439-42.  Back to cited text no. 2
Zach V, Bezov D, Lipton RB, Ashina S. Headache associated with moyamoya disease: A case story and literature review. J Headache Pain 2010;11:79-82.  Back to cited text no. 3


  [Figure 1], [Figure 2], [Figure 3]


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