Neurol India Home 
 

LETTERS TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 1  |  Page : 155--157

Neurologic immune reconstitution inflammatory syndrome in a seropositive patient: An interesting case

Neetu Ramrakhiani1, Vineet Mishra2,  
1 Department of Neurology, Fortis Escorts Hospital, Jaipur, Rajasthan, India
2 Department of Radiology, Vardhman Imaging Centre, Jaipur, Rajasthan, India

Correspondence Address:
Vineet Mishra
Department of Radiology, Vardhman Imaging Centre, Jaipur, Rajasthan
India




How to cite this article:
Ramrakhiani N, Mishra V. Neurologic immune reconstitution inflammatory syndrome in a seropositive patient: An interesting case.Neurol India 2016;64:155-157


How to cite this URL:
Ramrakhiani N, Mishra V. Neurologic immune reconstitution inflammatory syndrome in a seropositive patient: An interesting case. Neurol India [serial online] 2016 [cited 2021 Oct 16 ];64:155-157
Available from: https://www.neurologyindia.com/text.asp?2016/64/1/155/173651


Full Text

Sir,

Immune reconstitution inflammatory syndrome (IRIS) describes an inflammatory disorder associated with paradoxical worsening of neurological status, following initiation of highly active antiretroviral therapy (HAART) in human immunodeficiency virus (HIV)-positive individuals.[1]

The patient is a 47-year old male who was diagnosed to be seropositive for HIV in 1999 but was asymptomatic, and not on any medication. He developed fever and cough without hemoptysis in June 2013 and was diagnosed to be having chest tuberculosis and started on antituberculous therapy (ATT) in September 2013 along with the simultaneous administration of HAART. The pretreatment CD 4+ cell counts were 96 cells/µL. His pulmonary status and constitutional symptoms improved. He discontinued his ATT and HAART in November 2013, which was followed by clinical deterioration. Therefore the treatment was again retarted in January 2014. The patient was on isoniazid, rifampicin, pyrazinamide, and ethambutol as his initial antituberculous treatment and later, mofifloxacin was added. His HAART included efavirenz, zidovudine, and lamivudine.

In January 2014, he developed dysarthria and ataxia which had an insidious onset and a steady progression. Contrast-enhanced magnetic resonance imaging (CEMRI) revealed focal areas of altered signal intensity in the cerebellar hemisphere, pons, and medulla which were hyperintense on T2- and fluid-attenuated inversion recovery (FLAIR) images but without contrast enhancement. The possibility of progressive multifocal leucoenceplalopathy (PML) was kept [Figure 1]a and [Figure 1]b. His cerebrospinal fluid examination (CSF) showed normal proteins and sugar and negative stains for various organisms [Table 1].{Figure 1}{Table 1}

He had further deterioration leading to an altered sensorium and both urinary and faecal incontinence. The CD4 cell count was 172 cells/µL. Routine hematological investigations revealed a mild anemia (10.3 g%) but his biochemistry profile was normal. A repeat contrast enhanced magnetic resonance imaging showed T2 and FLAIR hyperintensities in bilateral cerebellar hemisphere, right middle cerebellar peduncle, pons, and medulla. Compared to the previous scans, progression of nonenhancing lesions was noticed in the pons and medulla. The repeat CSF fluid examination was normal [Table 1], with negative staining for various organisms including the JC/BK virus. In view of the radiological evidence of an increase in the number of brain lesions, his simultaneous clinical deterioration, and the negative CSF staining, the possibility of neuro-IRIS was kept. Patient was empirically started on steroids (methyprednisolone 1 g intravenously for 5 days followed by oral steroids which were continued for 6 weeks) and ATT was also given. From a progressive downhill course, he had sustained improvement. Over a period of next 4 to 5 days, he regained continence for urine and stool, had improvement in dysarthria, and could walk with minimal support. At the second follow-up, he could walk unsupported. His subsequent MRI was repeated which showed considerable regression of the lesion [Figure 2]. At a follow-up of 1 year and 3 months, the patient had only minimal residual dysarthria.{Figure 2}

In the above case, the patient had an ongoing tuberculous infection in the lung parenchyma and lymph nodes. Reintroduction of antituberculous treatment and HAART led to the development of neurologic symptoms within 2 months. Despite introduction of HAART, the patient had a progressive downhill course until steroids were added. Within days of addition of steroids, the patient started showing clinical improvement, which is sustained till date. The other differential diagnoses with such lesions include progressive multifocal leucoencephalopathy (PML); lymphoma; infection by toxoplasma, varicella-zoster virus, herpes simplex virus, and cytomegalovirus; HIV encephalitis; or, acute disseminated encephalomyelitis. Most of them were excluded based on the radiological findings, negative cerebrospinal fluid stains, and the improving trend in CD4 counts. A brain biopsy was not done in this case because of the sustained clinical improvement, potential hazards of the procedure, and brainstem and cerebellar location of the lesions. His ATT has been stopped and he continues to be on HAART. He most likely has neuro-IRIS without coinfection although it could be argued that in the post-HAART era, the sensitivity rate of CSF JC/BK virus in PML is around 57.5% according to one study.[2] Among the Indian studies, the mean survival of patients with PML is around 8 months.[3] Only time can give the final answer. A proper identification of the disease and maintaining a high index of suspicion are important factors in the successful management of neuro-IRIS. Although these manifestations are a result of upgrading of immunity due to the addition of HAART, under usual circumstances, stopping the HAART is not recommended as these conditions are often self-limiting. This case highlights the need to consider neurological immune reconstitution inflammatory syndrome as a differential diagnosis in those cases where a temporal relationship of the clinical deterioration is linked to the upgrading of immunity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1DeSimone JA, Pomerantz RJ, Babinchak TJ. Inflammatory reactions in HIV-1-infected persons after initiation of highly active antiretroviral therapy. Ann Intern Med 2000;133:447-54.
2Marzocchetti A, Di Giambenedetto S, Cingolani A, Ammassari A, Cauda R, De Luca A. Reduced rate of diagnostic positive detection of JC virus DNA in cerebrospinal fluid in cases of suspected progressive multifocal leukoencephalopathy in the era of potent antiretroviral therapy. J Clin Microbiol 2005;43:4175-7.
3Sharma SK, Soneja M, Ranjan S, Miglani S, Hari S, Sinha S, et al. Progressive multifocal leucoencephalopathy in HIV/AIDS: Observational study from a tertiary care centre in northern India. Indian J Med Res 2013;138:72-7.