Asmus, F.

Bötzel, Kai

Pfister, H. W.

Seelos, Klaus

Sporer, B.

English

Open Access LMU ( Ludwig-Maximilians-Univ. München)

Short Reports 96 Eur Neurol 2005;53:96–97  DOI: 10.1159/000085507  Posterior Fossa Tremor Induced by HIV-Associated Progressive Multifocal Leukoencephalopathy  B. Sporer   a , K. Seelos   b , F. Asmus   c , K. Boetzel  a , H.-W. Pfi ster   a   Departments of  a   Neurology,  b   Neuroradiology, Ludwig Maximilians University,  Munich ,  c   Department of Neurology, University of Tübingen,  Tübingen , Germany  Introduction  Movement disorders occur in 2–3% of patients with AIDS and are mostly caused by toxoplasmosis abscesses  [1] . However, other rare etiologies such as progressive multifocal leukoencephalopathy (PML) or human immunodefi ciency virus (HIV)-associated de-mentia have been described  [2, 3] . The diagnosis of PML is sup-ported by typical MRI fi ndings and the detection of JC virus (JCV) DNA in the cerebrospinal fl uid (CSF)  [4] . The lesions are mostly located in the parietal and occipital regions. Isolated infratentorial PML lesions have been reported, but they are a very rare fi nding in HIV-infected patients  [5] . We report on an AIDS patient who pre-sented with right-sided posterior fossa tremor in all probability due to an isolated infratentorial PML lesion.  Case Report  A 35-year-old HIV-infected German man presented with a slowly progressive unstableness and diplopia. Three months later, the physical examination showed a persistent rest, postural and kinetic tremor of the right hand, bilateral saccadic eye movements and a dissociated gaze nystagmus to the right, without further neu-rological defi cits. His CD4 cell count was low (40 cells/ m l), but in-creased (110 cells/ m l) after the antiretroviral medication was close-ly monitored.  Neurophysiological, CSF and MRI Findings  Electroencephalographic examination and nerve conduction studies were normal. Acoustic evoked potentials showed a reduc-tion of peak V during right-sided stimulation. Magnetic stimulation of the cerebral cortex revealed an increased central motor latency to the right arm (right 18.3 ms, left 8.1 ms). Tremor analysis showed a 4- to 5-Hz tremor ( fi g. 1 ).  CSF routine laboratory parameters were normal. Initially, CSF JCV (600 copies/ml) and BK virus (BKV; 200 copies/ml) had been detected by polymerase chain reaction (PCR) (real-time PCR with taqman probes). Three months later, CSF JCV PCR was negative, whereas the BKV load had increased to 1,500 copies/ml. Other CSF examinations including the assessment of an infection with  Trepo-nema pallidum, mycobacteria,  Cryptococcus neoformans, cytomeg-alovirus, varicella zoster and  Epstein-Barr virus were negative. CSF HIV load was low (140 copies/ml). Brain MRI revealed bilateral hyperintensities without contrast enhancement in the fl uid-attenu-ated inversion recovery (FLAIR) sequence with localization in the mesencephalon, the posterior pons, cerebellar peduncles and slight-ly in the white matter of the cerebellum, as well as a slight cerebel-lar atrophy ( fi g. 2 ).  Discussion  The tremor in our patient was a posterior fossa tremor in all probability due to an infratentorial PML lesion. Recent studies showed that lesions of different etiology with location in the poste-rior fossa can induce low and high frequency tremor  [6] .   The etiology of the lesion was in all probability PML, because initial CSF analysis by PCR detected JCV DNA, which is a very specifi c and sensitive parameter. Some features of PML are unique in our patient. PML with an isolated manifestation in the posterior fossa is a very rare fi nding in AIDS patients, and only a few cases of PML, predominantly affecting the cerebellum, have been de-scribed in patients without immunodefi ciency  [7] , hematological malignancies or AIDS  [5] . JCV infection of cerebellar granule cell neurons was recently shown by Du Pasquier et al.  [8]  in a HIV-in-fected patient, suggesting a role for JCV in cerebellar atrophy dur-12431 s Fig. 1. Tremor analysis. Bipolar surface re-cording with electrodes at a distance of 4 cm on the extensor digitorum communis mus-cle right (line 1) and left (line 3) and the fl exor digitorum superfi cialis muscle right (line 2) and left (line 4). The investigation was done with the Nicolet Viking IV (Nico-let Inc., Kleinostheim, Germany); vertical point-to-point distance indicates an ampli-tude of 200  m V. Line 1 shows rhythmic muscle activity with a frequency of approx-imately 4.5–5 Hz.  Short Reports 97ing HIV infection. The simultaneous detection of CSF BKV DNA by PCR and its increase in the control analysis may favor BKV as an (additional) etiological agent. Single cases of HIV-associated BKV-induced neurological disease reported a subacute meningo-encephalitis in combination with other systemic manifestations and a fatal clinical outcome  [9] . In contrast, our patient stabilized despite increased CSV BKV load. Thus, in our opinion, BKV infec-tion does not seem to play an important role in our patient.  During the observation period of 6 months and optimized an-tiretroviral therapy, neurological defi cits lessened, CD4 cell count rose and CSF JCV load became negative. Likewise, benefi cial ef-fects of antiretroviral therapy on the survival of AIDS patients with PML have been reported, particularly if symptom progression was halted and CSF JCV load was under control  [10] .  All in all, our case demonstrates a rare manifestation of HIV-associated PML. We conclude that PML should be considered even in isolated atypical lesions in the posterior fossa in patients with AIDS.  Acknowledgement  We thank Ms. J. Benson for editing the manuscript.   Fig. 2. Brain MRI shows bilateral hyperintensities (arrows) in the mesencephalon, the posterior pons, the cerebellar peduncles and slight-ly in the cerebellum white matter, as well as a slight cerebellar atrophy (FLAIR sequence). No contrast enhancement was observed.  References  1 Nath A, Jankovic J, Pettigrew LC: Movement disorders and AIDS. Neu-rology 1987;  37:  37–41.  2 Piccolo I, Causarano R, Sterzi R, Klopstock T, Sberna M, Oreste PL, Moioli C, Caggese L, Girotti F: Chorea in patients with AIDS. Acta Neu-rol Scand 1999;  100:  332–336.  3 Sporer B, Linke R, Seelos K, Paul R, Klopstock T, Pfi ster HW: HIV-in-duced chorea: Evidence for basal ganglia dysregulation by SPECT. J Neu-rol 2005;252:356–358.  4 Weber T, Klapper PE, Cleator GM, Bodemer M, Luke W, Knowles W, Cinque P, Van Loon AM, Grandien M, Hammarin AL, Ciardi M, Bogda-novic G: Polymerase chain reaction for detection of JC virus DNA in ce-rebrospinal fl uid: A quality control study. European Union Concerted Ac-tion on Viral Meningitis and Encephalitis. J Virol Methods 1997;  69:  231–237.  5 Whiteman ML, Post MJ, Berger JR, Tate LG, Bell MD, Limonte LP: Pro-gressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: Neuroimaging with clinical and pathologic correlation. Radiology 1993;  187:  233–240.  6 Vengud E, Jacquy J, Vanderkelen B, Manto MU: High-frequency synchro-nous bursts fi ring associated with asynchronous midbrain tremor. Rev Neurol (Paris) 2001;  157:  682–687.  7 Arai Y, Tsutsui Y, Nagashima K, Shinmura Y, Kosugi T, Wakai M, Ni-shikage H, Yamamoto J: Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodefi ciency. Neuropa-thology 2002;  22:  48–56.  8 Du Pasquier RA, Corey S, Margolin DH, Williams K, Pfi ster LA, De Gi-rolami U, Mac Key JJ, Wuthrich C, Joseph JT, Koralnik IJ: Productive infection of cerebellar granule cell neurons by JC virus in an HIV+ indi-vidual. Neurology 2003;  61:  775–782.  9 Vallbracht A, Lohler J, Gossmann J, Gluck T, Petersen D, Gerth HJ, Gen-cic M, Dorries K: Disseminated BK type polyomavirus infection in an AIDS patient associated with central nervous system disease. Am J Pathol 1993;  143:  29–39.  10 De Luca A, Giancola ML, Ammassari A, Grisetti S, Paglia MG, Gentile M, Cingolani A, Murri R, Liuzzi G, Monforte AD, Antinori A: The effect of potent antiretroviral therapy and JC virus load in cerebrospinal fl uid on clinical outcome of patients with AIDS-associated progressive multifo-cal leukoencephalopathy. J Infect Dis 2000;  182:  1077–1083.   Dr. B. Sporer, Department of NeurologyLudwig Maximilians University, Marchioninistrasse 15DE–81377 Munich (Germany)Tel. +49 89 7095 5821, Fax +49 89 7095 6673E-Mail bsporer@nefo.med.uni-muenchen.de 

Antinori A: The effect of potent antiretroviral therapy and JC virus load in cerebrospinal ﬂ uid on clinical outcome of patients with AIDS-associated progressive multifocal leukoencephalopathy.

Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeﬁ ciency. Neuropathology

Bogdanovic G: Polymerase chain reaction for detection of JC virus DNA in cerebrospinal ﬂ uid: A quality control study. European Union Concerted Action on Viral Meningitis and Encephalitis. J Virol Methods

Disseminated BK type polyomavirus infection in an AIDS patient associated with central nervous system disease.

Girotti F: Chorea in patients with AIDS. Acta Neurol Scand

High-frequency synchronous bursts ﬁ ring associated with asynchronous midbrain tremor. Rev Neurol (Paris)

Koralnik IJ: Productive infection of cerebellar granule cell neurons by JC virus in an HIV+ individual. Neurology

LC: Movement disorders and AIDS. Neurology

Limonte LP: Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: Neuroimaging with clinical and pathologic correlation. Radiology

Pﬁ ster HW: HIV-induced chorea: Evidence for basal ganglia dysregulation by SPECT.

Posterior fossa tremor induced by HIV-associated progressive multifocal leukoencephalopathy

B. Sporer

K. Seelos

F. Asmus

K. Boetzel

H.-W. Pfister

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Posterior Fossa Tremor Induced by HIV-Associated Progressive Multifocal Leukoencephalopathy

Universität München: Elektronischen Publikationen

https://epub.ub.uni-muenchen.de/16799/1/10_1159_000085507.pdf

Posterior fossa tremor induced by HIV-associated progressive multifocal leukoencephalopathy

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Universität München: Elektronischen Publikationen