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Jasmit Singh, Hrushikesh Kharosekar, Vernon Velho, Deepak Palande Department of Neurosurgery, Sir Jamshedjee Jeejeebhoy Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India E‑mail: [email protected] Access this article online Quick Response Code:

Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144484

Received: 14-03-2014 Review completed: 15-04-2014 Accepted: 03-10-2014

Cerebral venous thrombosis masquerading as hemicrania continua Figure 1: Right tranverse sinus thrombosis (black arrows)

Sir, Hemicrania continua (HC) is an idiopathic, chronic disorder characterized by a continuous, strictly unilateral headache associated with ipsilateral cranial autonomic symptoms. The symptoms of HC typically respond to indomethacin therapy.[1] This report presents a patient with cerebral venous thrombosis (CVT) who presented with indomethacin responsive hemicrania, probably the first such case. A 42‑year‑old man presented with persistent daily strictly right hemicranial headache of 1month duration. There were no pain‑free periods. Headache was of moderate to severe in intensity with superimposed paroxysms of short‑lasting (duration of up to 30 s) shooting pain in the same area. During exacerbations he would have conjunctival injection and nasal congestion on right side. Physical and neurological examination was normal. He was initially diagnosed to have hemicrania continua and was started on indomethacin 25 mg three times a day with complete resolution of headache within 24 h. After 2 weeks of therapy he had recurrence of headache whenever he missed indomethacin. He was further evaluated with magnetic resonance imaging (MRI) of brain which showed thrombosis of the right transverse sinus [Figure 1]. MR‑venography confirmed the absence of flow in the right transverse sinus. He was started on anticoagulation and antiedema measures and indomethacin was stopped. He had no further headaches after 3 days and is currently asymptomatic. 556

Initially considered a rare disorder, HC is being increasingly recognized[2] and is now classified under trigeminal autonomic cephalalgias according to ICHD 3 Beta 2013.[3] Our patient has satisfied all the criteria for the HC except for the duration of headache, for the diagnosis of primary HC the duration should be 3 months. Secondary headaches mimicking primary HC (including response to indomethacin) have been reported. They include internal carotid artery dissection, unruptured aneurysm, pineal cyst, pituitary tumor, ipsilateral mesenchymal tumor of the sphenoidal bone involving the clinoid process at the base of the skull, lung adenocarcinoma, and pontine stroke. Headache is by far the most frequent initial (80-90%) symptom of CVT, which has no specific characteristics. Most often it is diffuse, progressive, severe, and associated with other signs of intracranial hypertension. Cluster‑like headache secondary to CVT has been reported.[4] Patients who present with a clinical picture mimicking HC should be evaluated with a high index of suspicion for a possible CVT, even when the headache is responsive to indomethacin as it is associated with significant mortality and morbidity if not diagnosed and treated early. A secondary cause for HC should be considered when the duration of headache is shorter than 3 months.

Thomas Mathew, Sagar Badachi, G. R. K. Sarma, Raghunandan Nadig Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5

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Department of Neurology, St. John’s Medical College Hospital, Bengaluru, Karnataka, India E‑mail: [email protected]

References 1.

Pareja JA, Antonaci F, Vincent M. The hemicrania continua diagnosis. Cephalalgia 2001;21:940‑6. 2. Peres MF, Silberstein SD, Nahmias S, Shechter AL, Youssef I, Rozen TD, et al. Hemicrania continua is not that rare. Neurology 2001;57:948‑51. 3. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013;33:629‑808. 4. Park KI, Chu K, Park JM, Kim M. Cluster‑like headache secondary to cerebral venous thrombosis. J Clin Neurol 2006;2:70‑3. Access this article online Quick Response Code:

Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144486

Received: 04-07-2014 Review completed: 17-07-2014 Accepted: 25-09-2014

There was no past history of psychiatric illness. He had no perception of light in left eye and visual acuity was 3/60 in the right eye. Fundus revealed florid papilledema with haemorrhage and macular exudates. Perimetry revealed enlarged blind spot with concentric constriction of visual fields. There were no other neurological deficits. Computed tomography (CT) brain showed hyper dense superior sagittal sinuses with empty delta sign. [Figure 1a and b]. Magnetic resonance imaging  (MRI) showed filling defect in superior sagittal sinus and right transverse sinus and loss of flow void in superior sagittal sinus in T2. MR‑venogram revealed thrombosis of superior sagittal sinus, right transverse and straight sinus and few cortical veins [Figure 2b]. Repeat imaging (10 days after onset) showed additional appearance of parenchymal bleed in right inferior frontal gyrus, left temporal region and multiple micro bleeds in susceptibility weighted images. [Figure 3a‑c]. Resting functional MR‑images (fMRI) were acquired using a 3T scanner (Skyra, Siemens, Erlangen, Germany). For design specification and model of the fMRI the patient was asked to press the start response button at the onset of hallucination and press the end response button at the end of hallucination so as to record the

Charles Bonnet syndrome in a case of cerebral venous thrombosis with fMRI‑EEG correlation Sir, Charles Bonnet syndrome (CBS) is characterised by complex visual hallucinations with preserved insight in a patient with impaired vision or organic brain pathology. Occipital cortical and other cerebral resections, stroke, multiple sclerosis and temporal arteritis are the organic brain diseases associated with CBS. CBS in cerebral venous thrombosis (CVT) is not yet documented in English literature. A 27‑year‑old labourer presented with 4 days history of headache, recurrent vomiting and gradual onset of painless bilateral vision loss. He complained of seeing transient images of vivid human figures like men in uniform, lady combing her hair, lady standing with a shawl, a crying baby, landscapes, buildings and also animals like cat. They lasted only for few seconds to minutes and occurred infrequently almost daily. Patient was aware these were non‑existent. They occurred more often in dim light and in evenings. The complex visual hallucinations (VH) disappeared on looking at them and when someone talked to him. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5

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b Figure 1: (a and b) Hyper dense superior sagittal sinus in plain and empty delta sign on contrast scan respectively

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b Figure 2: (a) FMRI showing activation of bilateral (more on dominant side) parietal and temporal regions and (b) MR Venography showing non visualisation of superior sagittal sinus and right transverse sinus

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Cerebral venous thrombosis masquerading as hemicrania continua.

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