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 Table of Contents    
Year : 2019  |  Volume : 61  |  Issue : 3  |  Page : 315-316
Rapidly progressive dementia in a case of progressive multifocal leukoencephalopathy

1 Department of Psychiatry, NIMHANS, Bengaluru, Karnataka, India
2 Department of Psychiatry, Medical College, Kolkata, West Bengal, India
3 Department of Psychiatry, Government Medical College, Thiruvananthapuram, Kerala, India

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Date of Web Publication16-May-2019

How to cite this article:
Daimary PP, Das S, Chatterjee SS, Krishnapillai R. Rapidly progressive dementia in a case of progressive multifocal leukoencephalopathy. Indian J Psychiatry 2019;61:315-6

How to cite this URL:
Daimary PP, Das S, Chatterjee SS, Krishnapillai R. Rapidly progressive dementia in a case of progressive multifocal leukoencephalopathy. Indian J Psychiatry [serial online] 2019 [cited 2021 Oct 22];61:315-6. Available from:


Rapidly progressive dementia (RPD) has a dramatic departure from baseline cognition which often imposes a great challenge in finding the etiology. The most common causes of RPD are prion disease, Alzheimer's disease, acute encephalopathy, autoimmune disorder, etc. Progressive leukoencephalopathy (PML) is caused by the infection of oligodendrocytes by John Cunningham virus (JCV) in immunodeficient individuals.[1] Here, we present a case who showed rapid decline of Mini–Mental State Examination (MMSE) (17-4) in a span of 20 days, ultimately diagnosed to have progressive multifocal leukoencephalopathy with the underlying HIV.

   Case Report Top

A 46-year-old male with no prior psychiatric history presented with an insidious onset of forgetfulness and subtle mood changes leading to socio-occupational deficits. At initial assessment, he was found to have prosopagnosia, apraxia, and executive dysfunction. His MMSE was 17 out of 30 with significant impairment in orientation and recent memory. There was no history of sign of neurodeficit. His relevant blood investigations were unremarkable, but his magnetic resonance imaging (MRI) brain showed periventricular white-matter changes, with predominately involving the posterior part and brainstem. One ring-enhancing lesion was noted in the left temporal region, suggesting of tuberculoma. The condition was considered as progressive multifocal leukoencephalopathy (PMLC) with central nervous system (CNS) tuberculoma. He was found to be immunocompromised with HIV positivity (CD4 69). His cerebrospinal fluid protein was high with 180 mg/dl, but he was negative for Venereal Disease Research Laboratory or JCV DNA. JCV-polymerase chain reaction can be negative, especially in early PML as well as during immune reconstitution inflammatory syndrome (IRIS) despite typical clinical and radiologic findings.[2] His vasculitis profile was positive for perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA). Within the 20 days of inpatient care, the patient showed significant decline in cognitive functions, with scoring only 4 in the MMSE. The patient was then started on antiretroviral therapy (ART) and antitubercular treatment (rifampicin 600 mg, isoniazid 300 mg, ethambutol 800 mg, pyrazinamide 1500 mg, and Vitamin B6 40 mg). The patient was reviewed after 1 year, and there was significant improvement in his cognition. Previous studies had found that ART preserves or improves cognition in HIV-infected patients.[3] He started to go for his job with no recent memory impairment. This case emphasizes the early and correct diagnosis to reverse the condition like RPD.

   Discussion Top

PMLC is often associated with HIV, where dementia can be due to either of them or both. It is difficult to distinguish the primary causative factors as both can be the cause of CNS vasculitis with RPD.[4] In our case, the patient although did not have JCV positivity in the CSF, moderately increased protein level along with MRI findings is suggestive of CNS virus infection. The prognosis of PMLC has been improving with highly active antiretroviral therapy.[5] If left unmanaged, then the mortality rate is up to 50% within 3 months of diagnosis. Prolonged survival can be possible with early treatment initiation.[6],[7] In our case, the person probably had significant dissemination of infection or vasculitis (PNCA+) leading to rapid cognitive decline, which was surprisingly counteracted with the initiation of ART.[8] Patient's nearly normal socio-occupational function probes the importance of early diagnosis and treatment in a case of RPD which often presents to a psychiatrist with behavioral changes.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Geschwind MD, Haman A, Miller BL. Rapidly progressive dementia. Neurol Clin 2007;25:783-807, vii.  Back to cited text no. 1
Kuhle J, Gosert R, Bühler R, Derfuss T, Sutter R, Yaldizli O, et al. Management and outcome of CSF-JC virus PCR-negative PML in a natalizumab-treated patient with MS. Neurology 2011;77:2010-6.  Back to cited text no. 2
de Jager CA, Joska JA, Hoffman M, Borochowitz KE, Combrinck MI. Dementia in rural South Africa: A pressing need for epidemiological studies. S Afr Med J 2015;105:189-90.  Back to cited text no. 3
Zunt JR, Tu RK, Anderson DM, Copass MC, Marra CM. Progressive multifocal leukoencephalopathy presenting as human immunodeficiency virus type 1 (HIV)-associated dementia. Neurology 1997;49:263-5.  Back to cited text no. 4
Pavlovic D, Patera AC, Nyberg F, Gerber M, Liu M, Progressive Multifocal Leukoencephalopathy Consortium, et al. Progressive multifocal leukoencephalopathy: Current treatment options and future perspectives. Ther Adv Neurol Disord 2015;8:255-73.  Back to cited text no. 5
Brew BJ, Davies NW, Cinque P, Clifford DB, Nath A. Progressive multifocal leukoencephalopathy and other forms of JC virus disease. Nat Rev Neurol 2010;6:667-79.  Back to cited text no. 6
PML Consortium; 2017. Available from: [Last accessed on 2018 Dec 08].  Back to cited text no. 7
Dorsett M, Liang SY. Diagnosis and treatment of central nervous system infections in the emergency department. Emerg Med Clin North Am 2016;34:917-42.  Back to cited text no. 8

Correspondence Address:
Soumitra Das
Department of Psychiatry, NIMHANS, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/psychiatry.IndianJPsychiatry_76_18

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