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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 2  |  Page : 426-427

Rapidly progressive dementia with myoclonus: Not Creutzfeldt-Jakob disease

Department of Neurology, National Neurosciences Institute, Singapore

Date of Web Publication10-Mar-2017

Correspondence Address:
Dr. M Saini
National Neurosciences Institute, 11 Jalan Tan Tock Seng – 308433
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/neuroindia.NI_57_16

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How to cite this article:
Saini M, Varghese N M, Su Wai KH, Loh N K. Rapidly progressive dementia with myoclonus: Not Creutzfeldt-Jakob disease. Neurol India 2017;65:426-7

How to cite this URL:
Saini M, Varghese N M, Su Wai KH, Loh N K. Rapidly progressive dementia with myoclonus: Not Creutzfeldt-Jakob disease. Neurol India [serial online] 2017 [cited 2023 Mar 26];65:426-7. Available from: https://www.neurologyindia.com/text.asp?2017/65/2/426/201862

A 70-year-old lady presented with progressive memory disturbance, dressing difficulty, urinary incontinence, and gait ataxia over 12 weeks. There was no past history of stroke, stroke-like events or seizures, or measles in childhood. No history of headache was reported, and family history was unremarkable. Examination revealed generalized hypertonia, symmetrical and bilateral cog-wheeling, gait ataxia without limb dysmetria, enhanced jaw jerk, and hypersomnolence. Discrete, multi-focal, distal predominant myoclonus was noted in the upper limbs, which was enhanced on action; sensitivity to touch or startle was absent. Fundus examination did not show any evidence of pigmentary retinal changes or optic atrophy. Mini mental state examination (MMSE) score was 6/27. A clinical diagnosis of rapidly progressive dementia was made.

Magnetic resonance imaging (MRI) of the brain showed diffuse, symmetrical, white matter lesions [Figure 1]a, prominent veins in cerebral sulci/cerebellar fissures [Figure 1]b, and prominent vasculature at the region of vein of Galen/straight sinus. No arterial or venous infarcts were noted. Dural arteriovenous fistulae (DAVF) were confirmed on 4-vessel angiogram (straight sinus [near the vein of Galen], at torcula). Marked retrograde venous sinus drainage was seen into the superior sagittal sinus, right transverse sinus, superficial cortical veins, and deep venous system.
Figure 1: Magnetic resonance imaging of the brain showing symmetrical, confluent T2 hyperintensities in bilateral cerebral hemispheres (top panel, a). Gradient recall echo images (bottom panel, b) show prominent veins (hypointense, curvilinear signals; yellow arrows) in bilateral cerebellar fissures and cerebral sulci. No parenchymal hematoma or venous sinus thrombosis was noted. Computed tomography (CT) of the brain shows diffuse cerebral edema with effaced cerebral sulci and compression of frontal horns (c). Post-procedure CT scan shows the normal frontal horns, opening of Sylvian fissures, and better visualization of the cortical sulci (d)

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The patient underwent skeletonization of AVF, followed by embolization of persistent meningeal arterial feeders. Post-embolization improvement in cognition was noted (from 6 to 14/27). Hypersomnolence and myoclonus resolved completely after intervention.

Dementia in patients with DAVF has been attributed to venous hypertension, leading to white matter changes and venous hypertensive encephalopathy.[1],[2] Labeyrie et al., have shown that a reflux from the fistula into the straight and superior sagittal sinuses is a necessary condition to develop reversible cognitive deficits in DAVF.[2] Our patient presented with a rapid decline in cognition, associated with myoclonus and ataxia. In this scenario, other diagnoses that merit attention include Creutzfelt Jacob disease (CJD), Hashimoto's encephalopathy, subacute sclerosing panencephalitis (SSPE), and myoclonic epilepsies.[3] The post-embolization resolution of myoclonus and improvement in cognition (sustained over several weeks) argued against CJD and SSPE. A mild increase in serum thyroid stimulating hormone (with normal free thyroxine) was noted; however, improvement in neurological status occurred in the absence of thyroid hormone or steroid use. We feel that our patient was a case of rapidly progressive dementia, secondary to venous hypertension resulting from the presence of an extensive dural AVF. Consistent with the findings of Labeyrie et al., our patient had significant retrograde venous drainage into the superior sagittal sinus and the deep venous system. The clinical improvement following surgical procedures accompanied by a decrease in cerebral edema [Figure 1]c and [Figure 1]d is consistent with this diagnosis.

This case is illustrative of an unusual, and potentially treatable, cause of rapidly progressive dementia. Dementia secondary to DAVF(s) has been infrequently reported;[1],[2] its presentation mimicking CJD is exceedingly rare.[3],[4] Though rare, it is a potentially reversible cause of dementia that may be easily identified on MRI.

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 » References Top

Kwon BJ, Han MH, Kang HS, Chang KH. MR imaging features of intracranial dural arteriovenous fistulas: Relations with venous drainage patterns. AJNR Am J Neuroradiol 2005;26:2500-7.  Back to cited text no. 1
Labeyrie MA, Lenck S, Saint-Maurice JP, Bresson D, Houdart E. Dural arteriovenous fistulas presenting with reversible dementia are associated with a specific venous drainage. Eur J Neurol 2014;21:545-7.  Back to cited text no. 2
Geraldes R, Albuquerque L, Ferro JM, Sousa R, Sequeira P, Campos J. Rapidly progressive cognitive impairment, ataxia, and myoclonus: An unusual presentation of a dural arteriovenous fistula. J Stroke Cerebrovasc Dis 2012;21:619.  Back to cited text no. 3
Jagtap SA, Nair SS, Jain N, Nair MD. Rapidly progressive dementia, Parkinsonism and myoclonus: An unusual presentation of dural arteriovenous fistula. Neurol India 2014;62:107-10.  Back to cited text no. 4
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