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Table of Contents    
CASE REPORT
Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1018-1020

Acute Cerebellar Ataxia as the Presenting Symptom of Progressive Multifocal Leukoencephalopathy with HIV - A Case Report


Department of General Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Date of Submission06-Jun-2017
Date of Decision15-Jun-2017
Date of Acceptance08-Aug-2019
Date of Web Publication2-Sep-2021

Correspondence Address:
Kuldeep Singh
Department of General Medicine, Regional Institute of Medical Sciences, Imphal - 795 004, Manipur
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.325348

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 » Abstract 


A 45-year-old man presented with acute onset ataxia for last 1 week. On examination he had signs of left-sided cerebellar involement. MRI brain revealed asymmetric altered signal intensities in bilateral cerebellar hemispheres suggesting demyelinating lesions. ELISA for Human Immune Deficiency virus-1 was positive. CSF JC virus DNA PCR was positive. A diagnosis of Progressive Multifocal Leukoencephalopathy (PML) was made on the basis of clinico-radiological picture and JC virus DNA PCR presence in CSF. PML is unknown and under diagnosed CNS infection seen in HIV patients mostly seen with advanced disease. We present an unusual case report where isolated cerebellar involvement occurred as the first AIDS defining event in the absence of appreciable immunodeficiency in a patient with previously undiagnosed HIV infection.


Keywords: Cerebellar ataxia, HIV, JC virus, progressive multifocal encephalopathy
Key Message: HIV related PML due to JC Virus with isolated cerebellar involvement may rarely be the initial presenting feature.


How to cite this article:
Singh TS, Singh K. Acute Cerebellar Ataxia as the Presenting Symptom of Progressive Multifocal Leukoencephalopathy with HIV - A Case Report. Neurol India 2021;69:1018-20

How to cite this URL:
Singh TS, Singh K. Acute Cerebellar Ataxia as the Presenting Symptom of Progressive Multifocal Leukoencephalopathy with HIV - A Case Report. Neurol India [serial online] 2021 [cited 2021 Oct 18];69:1018-20. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1018/325348




HIV is a global menace with over 5 million people living with HIV/AIDS in India. AIDS virus is known to be a neurotropic virus and CNS involvement is seen as presenting complaint in 10% of cases, with a spectrum of neurological manifestations. Isolated cerebellar symptoms in HIV have been reported rarely and usually result from opportunistic infections.[1] Mechanisms of CNS involvement can be via demyelination due to autoimmunity elicited by viral proteins, secondary to lymphomas, secondary to cell-mediated immunodeficiency or other general and systemic complications of HIV. The prominent demyelinating CNS lesions reported in HIV are acute disseminated encephalomyelitis, multiple sclerosis like demyelination, and progressive multifocal leukoencephalpathy. We present a 45-year-old male with acute cerebellar involvement in the form of bilateral, asymmetrical demyelination as the first AIDS defining feature with previously undiagnosed HIV infection.


 » Case Report Top


A 45-year-old male presented to the neurology OPD with complaints of difficulty in walking for last 7 days, with tendency to fall on left side while trying to walk. He did not have history of nausea, vomiting, headache, loss of consciousness or any abnormal movements, facial deviation, nasal regurgitation of food, shortness of breath, or bowel bladder involvement. There was no history of head injury, fever, anorexia, or weight loss. The patient denied having vaccinations or upper respiratory infections in the recent past. There was no history of alcohol, drug abuse or previous illness and his family history was unremarkable. His appetite and sleep were normal. General physical examination was normal, higher mental functions, and cranial nerves were intact, motor examination power was normal bilaterally, mild hypotonia was present on left side in both upper and lower limb, planters were flexors bilaterally and there was no sensory loss. Speech was normal. On cerebellar examination dysdiadokochokinesia, past pointing, intention tremers and heel knee test was positive on left side and was not able to perform tandem walking. Fundoscopy was done which was unremarkable.

His complete blood counts, liver and kidney function tests and serum electrolytes were normal. On brain imaging, CT scan was normal but MRI revealed asymmetrical altered signal intensities in bilateral cerebellar hemispheres. The lesions were hypointense on T1 weighted images and hyperintense on T2 [Figure 1] and FLAIR suggestive of demyelination/ischaemia. Visual Evoked potential (VEP) and Brainstem Evoked Response Audiometry (BERA) were normal. To rule out multiple sclerosis (MS) and infections a cerebrospinal fluid (CSF) analysis was done which showed 4 cells, all neutrophils with 72 mg/dl sugar, 42 mg/dl proteins and oligoclonal bands were positive in both CSF and blood. A subsequent MR angiography of cerebral circulation was done which was normal. Taking into the account of his acute demyelinative illness with positive oligoclonal bands, the patient was prescribed pulse Methylprednisolone therapy (1 gram per day for 5 days) but no improvement was observed even after 1 week and patient continued to deteriorate and developed bilateral cerebellar signs. On further evaluation he was tested by ELISA for HIV that came out to be positive for type 1 virus and was subsequently confirmed by western blot assay. His CD4 counts were 69 cells/μL. A repeat lumbar puncture was done to rule out opportunistic infections and sample was sent for JC virus DNA PCR that came out to be positive. Repeat MRI showed significant increase in the size of the lesion bilaterally. On the basis of patient's clinical and radiological picture and positive DNA PCR for JC Virus we formulated a probable diagnosis of PML. The patient was started on HAART but despite treatment, he showed progressive worsening, and later discharged against medical advice.
Figure 1: Repeat MRI Image D – Showing FLAIR images with increased hyperintensity in bilateral cerebellar hemisphere (Left > Right)

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 » Discussion Top


HIV is a neurotropic virus and CNS involvement is seen in approximately 10% cases of HIV. Neurological involvement in HIV can be due to opportunistic infections, drug toxicity, malignancy, cerebrovascular disease, or as a consequence of direct HIV infection. Neurological diseases associated with HIV-1 are becoming more common as antiretroviral therapy improves patient survival.[2] Demyelinating CNS disorders in HIV are rare and are due to acute multiple sclerosis like leukoencephalopathy,[3] PML,[4] acute disseminated encephalomyelitis.[5]

Multiple sclerosis has a female preponderance, presents with sensory symptoms, optic neuritis and pyramidal signs. MRI reveals typical sharply demarcated lesions mostly in periventricular region, although oligoclonal bands is seen multiple sclerosis but they can also be present in HIV infection due to chronic antigenic stimulation leading to B-cell hyperplasia.[6] If MS is coincidently related to HIV infection it usually manifests at high CD4 counts as previously reported by others.[7] González-Duarte et al. described a single case in 2011 and reviewed 15 other case reports published between 1989 and 2009. The mean CD4 + cell count at first episode for 12 cases was 571 cells/mm.[3],[7] Overall HIV-1 have a protective effect on MS that was shown in a recent comparative cohort study conducted in the UK which reported a negative association of HIV and MS with a rate ratio of 0.38 (95% CI, 0.15 to 0.79) (Gold et al. 2015).[8] Considering all these MS was unlikely in our patient which was again supported by the poor response of symptoms to steroids rapid worsening of disease within days. So, after excluding Multiple sclerosis a diagnosis of PML was made.

PML is a progressive demyelinating disorder due to John Cunningham virus (DNA papovavirus) infection of the myelin-producing oligodendrocytes. The majority of patients with PML are believed to be infected with the JC virus in childhood or early adolescence; the virus remains latent in the CNS unless reactivated in the setting of immunodeficiency.[9] PML is found in approximately 5% of autopsies of patients who die of AIDS.[10] The greatest risk of developing PML occurs among patients with CD4 counts in the range of 50–100 cells/μL. PML typically results in a progressive neurologic decline, and patients develop cognitive impairment, altered mental status, and personality changes. Motor and sensory changes also occur, and the patients may develop seizures. Without treatment, patients have a progressive downhill course, with death occurring within 1 year of diagnosis of PML in 90% of cases.[11] Polymerase chain reaction testing of the cerebrospinal fluid for the John Cunningham virus assists in making the diagnosis, with a specificity approaching 96%.[12] The most frequently affected regions by PML are the cerebral hemispheres at the periventricular and/or subcortical white matter, followed by the cerebellum and brain stem.[13] The frontal and parieto-occipital lobes are classically affected. These lesions usually have high signal intensity on T2- weighted/FLAIR sequence and hypointense on T1- weighted images. Contrast enhancement is usually not observed and mass effect is uncommon.[13] A combination of characteristic clinical picture and typical imaging findings with positive CSF DNA PCR the diagnosis of Cerebellar PML was made. Although Brain biopsy carries is diagnostic investigation of choice it was not done due to technical difficulties. Only few cases of cerebellar PML had been described in literature[14],[15] in both these cases patient presented with combination of cerebellar features including Dysarthria, limb dysmetria, gait instability head tremor. Our patient also presented with cerebellar features initially unilateral but progressed rapidly to both sides. Later the patient was referred to ART centre for further management there his condition deteriorated and he died three months later. To conclude, it is imperative for the general physicians to be aware that HIV can present as subacute cerebellar involvement as the first AIDS defining illness in a relatively immunocompetent patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Tagliati M, Simpson D, Morgello S, Clifford D, Schwartz RL, Berger JR. Cerebellar demyelination associated with human immunodeficiency virus infection. Neurology 1998;50:244-25.  Back to cited text no. 1
    
2.
Skiest DJ. Focal neurological disease in patients with acquired immunodeficiency syndrome. Clin Infect Dis 2002;34:103-15.  Back to cited text no. 2
    
3.
Sardar P, Guha P, Roy D, Bandyopadhya D, Chatterjee SK. Multiple sclerosis like demyelination in early HIV infection-A rare presentation. JAID Clinic Res 2011;2:124.  Back to cited text no. 3
    
4.
Cinque P, Koralnik IJ, Gerevini S, Miro JM, Price RW. Progressive multifocalleukoencephalopathy in HIV-1 Infection. Lancet Infect C 2009;9:625-36.  Back to cited text no. 4
    
5.
Noorbaksh F, Johnson TR, Emery D, Power C. Acute disseminated encephalomyelitis: Clinical and pathogenesis features. Neurol Clin 2008;759-80.  Back to cited text no. 5
    
6.
Konstantinopoulos PA, Dezube BJ, Pantanowitz L, Horowitz GL, Beckwith BA. Protein electrophoresis and immunoglobulin analysis in HIV-infected patients. Am J Clin Pathol 2007;128:596-603.  Back to cited text no. 6
    
7.
González-Duarte A, Ramirez C, Pinales R, Sierra-Madero J. Multiple sclerosis typical clinical and MRI findings in a patient with HIV infection. J Neurovirol 2011;17:504-8  Back to cited text no. 7
    
8.
Gold J, Goldacre R, Maruszak H, Giovannoni G, Yeates D, Goldacre M. HIV and lower risk of multiple sclerosis: Beginning to unravel a mystery using a record-linked database study. J Neurol Neurosurg Psychiatry 2015;86:9-12.  Back to cited text no. 8
    
9.
HoganTF, Padgett BL, Walker DL, Borden EC, McBain JA. Rapid detection and identification of JC virus and BK virus in human urine by using immunofluorescence microscopy. J Clin Microbiol 1980;11:178-83.  Back to cited text no. 9
    
10.
Petito CK, Cho ES, Lemann W, Navia BA, Price RW. Neuropathology of acquired immunodeficiency syndrome (AIDS): An autopsy review. J Neuropathol Exp Neurol 1986;45:635-46.  Back to cited text no. 10
    
11.
Berger JR, Levy RM, Flomenhoft D, Dobbs M. Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy. Ann Neurol 1998;44:341-9.  Back to cited text no. 11
    
12.
McGuireD, Barhite S, Hollander H, Miles M. JC virus DNA in cerebrospinal fluid of human immunodeficiency virus-infected patients: Predictive value for progressive multifocal leukoencephalopathy. Ann Neurol 1995;37:395-9.  Back to cited text no. 12
    
13.
Atlas SW. Progressive multifocal leukoencephalopathy. In: Atlas SW, editor. Magnetic Resonance Imaging of Brain and Spine. 4th ed. Lippincott Williams and Wilkins; 2008. p. 949-55.  Back to cited text no. 13
    
14.
Kastrup O, Wanke I, Esser S, Maschke M. Evolution of purely infratentorial PML under HAART-negative outcome under rapid immune reconstitution. Clin Neurol Neurosurg 2005;107:509-13.  Back to cited text no. 14
    
15.
Svensson PA, Larsson EM. Infratentorial progressive multifocal leukoencephalopathy (PML) in a patient with SLE. Eur Radiol 2008;18:1526-8.  Back to cited text no. 15
    


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