7. Suzuki S. Detection of latent herpes simplex virus in human vestibular ganglia.Hokkaido Igaku Zasshi 1996; 71: 561–571.

8. Levitz RE. Herpes simplex encephalitis: A review. Heart Lung 1998; 27: 209.

Protracted mumpsencephalitis with goodoutcomeq

Peter Kim MBBS(HONS)MBBS(HONS), Cecilia Cappelen-Smith PPhhDD

Department of Neurology and Neurophysiology, Liverpool Hospital, Sydney,

Australia

Summary There is limited published information regarding the out-

come of patients with prolonged encephalitis. This report details the

case of a patient with an encephalitic illness with a protracted period

of coma and a favourable outcome. Extensive investigation revealed

seroconversion for mumps infection. A household contact had

measles, mumps, rubella (MMR) vaccination 10 days prior to his

presentation.

ª 2005 Elsevier Ltd. All rights reserved.

Journal of Clinical Neuroscience (2005) 12(8), 959–961

0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved.

doi:10.1016/j.jocn.2004.11.013

Keywords: encephalitis, mumps, MMR vaccine

Received 5 July 2004

Accepted 15 November 2004

Correspondence to: Dr. Cecilia Cappelen-Smith PhD, Department of

Neurophysiology, Liverpool Hospital, Locked bag 7103, Liverpool BC, NSW

1871, Australia. Tel.: +61 2 9828 3646; Fax: +61 2 9828 3846;

E-mail: c.cappelensmith@unsw.edu.au

INTRODUCTION

Encephalitis may be mild and self-limiting or it may produce adevastating illness. Herpes simplex virus is the most commoncause of non-seasonal encephalitis in Australia. Magnetic reso-nance imaging (MRI) and cerebro-spinal fluid (CSF) examinationusually provide useful diagnostic information. Brain biopsy is nowseldom performed in the diagnosis of encephalitis, restricted tothose cases, which fail to respond to aciclovir. 1 Brain biopsywas performed in this case, and was non-contributory to diagnosis.This case represents a patient with a severe encephalitic illnessdue to primary mumps infection with a protracted course andfavourable outcome.

CASE REPORT

A previously well 35-year-old man presented following a wit-nessed seizure causing a motor vehicle collision. He had minorbruising but no significant injuries. There was no prior historyof epilepsy, and no prodromal illness, fever, vaccinations, recentoverseas travel or other past medical or psychiatric history. Hisson had been vaccinated 10 days prior for measles, mumps and ru-bella (MMR). Initial neurological examination was normal. Threedays after admission he became confused and aggressive. There

was no neck stiffness or focal neurological signs. Over the nexttwo days he became progressively drowsy and developed intracta-ble epileptic seizures, requiring transfer to the intensive care unit.He became unconscious and required intubation. He was given a14-day course of intravenous aciclovir and multiple anticonvul-sant agents. He developed a complex movement disorder involv-ing non-purposeful facial (lip smacking, grimacing, chewing) andlimb movements. During this period he was given intravenousmethylprednisolone 1g daily for 10 days. He remained in theintensive care unit for approximately 3 months.EEG and CT brain scan on admission were normal. Lumbar

puncture showed a white cell count of 100 · 106/L with lympho-cyte predominance, but normal protein and glucose. Polymerasechain reaction (PCR) on the CSF for herpes simplex virus, entero-virus, rabies and mycobacterium tuberculosis were negative. CSFviral and bacterial cultures were negative, as was cytology. HIVtesting was negative. Investigations excluded metabolic, toxicand inflammatory causes of encephalitic illness. Serological andPCR testing is summarized in Table 1. Convalescent serologyfor mumps virus showed seroconversion. Brain MRI with gadolin-ium showed mild to moderate signal change in both medial tem-poral lobes, the left posterior parietal and left medial occipitalregions.Repeat EEG showed marked slowing, consistent with a diffuse

encephalopathic process (Figs. 1 and 2). Repeat lumbar punctureshowed an elevated protein at 1.26 g/L, normal glucose and nor-mal white cell count. Repeat PCR on the CSF for HSV was neg-ative. Brain biopsy showed only mild non-specific reactive glialchanges with no abnormalities of the dura or arachnoid. PCR onthe brain biopsy was negative (Table 1).He required intensive supportive care with tracheostomy and a

percutaneous gastrostomy tube for feeding, remaining in a chronicvegetative state for 6 months. His level of consciousness then

Table 1 Summary of serological testinga

Infective agent Serology CSF PCR Brain biopsy

PCR

Measles PAST

Rubella PAST

Ebstein-Barr virus PAST

Cytomegalovirus PAST

Herpes simplex virus PAST NEG · 2 NEG

Herpes zoster virus PAST

Human Herpes 6 virus PAST

Mumps SERO

Cryptococcal antigen NEG NEG

Mycoplasma NEG

Legionella pneumophilia NEG

Chlamydia NEG

Toxoplasmosis NEG

Australian bat lyssavirus NEG NEG

Adenovirus NEG

Influenza A & B NEG

Hepatitis B & C NEG

Lymes disease NEG

Cat scratch disease NEG

Q fever NEG

Flavivirus (Murray Valley,

Dengue, Kunjin)

NEG

Hendravirus NEG

HIV NEG · 2

Mycobacterium tuberculosis NEG NEG

Enterovirus (Cox B 1-6, Cox A9,

Echo 4,6,9,14,24,30)

NEG NEG

Syphilis NEG NEG

Rickettsial NEG

PAST = past exposure, SERO = seroconversion, NEG = negative, PCR =

polymerase chain reaction.a Acute and convalescent specimens taken.

qThis paper was presented on the 11th May, 2004 at the Australian

Association of Neurologists Annual Scientific Meeting, Perth, Australia.

ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(8)

959Protracted mumps encephalitis with good outcome

began to improve. By 8 months he was able to follow simple com-mands. He gradually gained mobility and independence in activi-ties of daily living. At 14 months he had short-term memorydeficits, but was retraining to re-enter the work force.

DISCUSSION

Our patient presented with features of encephalitis including: pro-gressive deterioration in level of consciousness; psychiatric distur-bance; intractable seizures; and a complex movement disorder.2,3

Extensive investigations were performed to find the cause ofencephalitis. The only positive result was seroconversion formumps virus, indicating the most likely cause was encephalitisdue to primary mumps infection. Interestingly, but most likelycoincidentally, a household contact had received recent MMRvaccination.Mumps infection is often a mild illness that commonly affects

children and adolescents.4 Encephalitis complicating primarymumps infection in the adult is extremely uncommon.2 In Finland,the incidence of viral encephalitis was estimated to be 14 permillion adults per year, of which 4% were considered secondary

to mumps infection, with HSV (as in Australia) the most commonetiologic agent.2 Ideally PCR for mumps should have been per-formed on CSF in this case, but was not available at the time ofpresentation. It is in such cases that storage of CSF for further test-ing as such tests become available is recommended.In Australia, two live attenuated MMR vaccine preparations are

available (M-M-R II�, MerckSharp&Dohme and Priorix�, Glaxo-SmithKline).5 They are safe and effective and have resulted in adramatic reduction in encephalitides associated with theseviruses.6 Although MMR vaccine-related cases of meningitis havebeen reported, they are not known to be transmitted from vacci-nees to contacts. It is safe to vaccinate household contacts ofimmunosuppressed patients with these agents.5–7 To our knowl-edge, only live attenuated polio and smallpox vaccines have beennoted to spread from vaccine recipients to their contacts and thenonly very rarely cause symptomatic disease.3,8 Postvaccinalencephalopathy and encephalitis are well recognised, but rare,complications of smallpox vaccination.9 This postinfectious syn-drome usually occurs 7–14 days following vaccination.9

This case is unusual in that there was a very severe and pro-tracted illness, where the patient was in a chronic vegetative state

Fig. 1 EEG day 10 shows diffuse slowing consistent with encephalopathy.

Fig. 2 EEG showing chewing artefact due to movement disorder.

Journal of Clinical Neuroscience (2005) 12(8) ª 2005 Elsevier Ltd. All rights reserved.

960 Kim and Cappelen-Smith

for 6 months, before a gradual but dramatic clinical improvement.Our report highlights that when the natural history of the enceph-alitis is uncertain, long-term intensive support is indicated.10

REFERENCES

1. Bearman MH, Wesselingh SL. Acute community acquired meningitis andencephalitis. MJA 2002; 176: 389–396.

2. Rantalaiho T, Farkkila M, Vaheri A, Koskiniemi M. Acute encephalitis from1967 to 1991. J Neurol Sci 2001; 184: 169–177.

3. Miravalle A, Roos KL. Encephalitis complicating Smallpox vaccination. ArchNeurol 2003; 60: 925–928.

4. Galbraith NS, Young SE, Pusey JJ, et al. Mumps surveillance in England andWales 1962–81. Lancet 1984; 1: 91–94.

5. The Commonwealth of Australia. The Australian Immunisation Handbook, 8th

Edition 2003. National Health & Medical Research Council; National CapitalPrinters, Canberra. pp. 153–154.

6. Koskiniemi M. Vaheri A. Effect of measles, mumps, rubella vaccination onpattern of encephalitis in children. Lancet 1989; 1: 31–34.

7. Furesz J. Safety of Live Mumps Virus Vaccines. J Med Virology 2002; 67:299–300.

8. Kew O, Morris-Glasgow V, Landaverde M, et al. Outbreak of Poliomyelitis inHispaniola Associated with Circulating Type 1 Vaccine-Derived Poliomyelitis.Science 2002; 296: 356–359.

9. Cono J, Casey CG, Bell DM. Smallpox vaccination and adverse reactions.Guidance for clinicians. MMWR Recomm Rep 2003; 52: 1–28.

10. Frasca J, Kilpatrick TJ, Burns RJ. Protracted form of encephalitis with goodoutcome. MJA 1993; 158: 629–630.

Unusually long survival in acase of medullomyoblastoma

Awadhesh Kumar Jaiswal1 MCMChh, Sushila Jaiswal2 MDMD,

Ashok Kumar Mahapatra1 MCMChh, Mehar Chand Sharma3 MDMD

1Departments of Neurosurgery, Neurosciences Centre, All India Institute of

Medical Sciences, Ansari Nagar, 2Department of Pathology, Lady Harding’s

Medical College, 3Neuropathology, Neurosciences Centre, All India Institute of

Medical Sciences, Ansari Nagar; New Delhi, India

Summary Medullomyoblastoma is a rare variant of medulloblas-

toma containing a component of striated and smooth muscle with

survival of 3 to 4 years following surgery and radiotherapy. The

authors report a rare case of medullomyoblastoma with an unusual

survival of over 11 years without recurrence. The relevant literature is

briefly reviewed.

ª 2005 Elsevier Ltd. All rights reserved.

Journal of Clinical Neuroscience (2005) 12(8), 961–963

0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved.

doi:10.1016/j.jocn.2004.11.019

Keywords: medulloblastoma, medullomyoblastoma, survival

Received 5 August 2004

Accepted 15 November 2004

Correspondence to: Dr Awadhesh Kumar Jaiswal, 19/162, Basti Sarai Rohilla

Old Rohtak Road, Delhi-110035, India. Tel.: +91 011 661123;

Fax: +91 011 686 2663;

E-mail: jaiswal_dr_2002@yahoo.co.in

INTRODUCTION

Medullomyoblastoma (MMB) was first described by Marinescoand Goldstein in 1935 and is considered a primitive neuroectoder-mal tumor (PNET) having a component of striated and smooth

muscle and is thought to be a variant of malignant teratoma or ateratoid tumor with poor outcome despite surgery and radiother-apy.1–4 Less than 40 cases have been reported in the literatureto date with maximum survival being 3 to 4 years, even after sur-gical excision and radiotherapy.5,6 We report a case of MMB witha survival time of over 11 years post-surgery and radiotherapy,with no recurrence.

CASE REPORT

An 8 year-old boy presented to us in 1991 with a history of pro-gressive headache and vomiting for one month and unsteadinessof gait for 15 days.General and systemic examination was normal. Neurological

examination revealed normal higher mental function. Visual acu-ity was 6/6 bilaterally. Fundus examination showed bilateral grosspapilloedema. He had bilateral horizontal nystagmus. There wasno motor or sensory deficit but he had bilateral cerebellar signswith gait ataxia. CT scan revealed a contrast enhancing, mixeddensity mass in the vermis of the cerebellum with compressionof the fourth ventricle, causing obstructive hydrocephalus(Fig. 1). A right ventriculo-peritoneal shunt was inserted the nextday, following which his headache and vomiting improved. Threedays later he underwent a midline suboccipital craniectomy andtumor excision. Intraoperatively, the tumor was large, reachingto the surface of the cerebellum and causing widening of the ver-mis. It was a soft, vascular, grayish tumor, easily removed withsuction and with a good plane of cleavage between the cerebellumand the IVth ventricle. Gross total excision was achieved.

Histopathology

Histopathological examination of the tumor specimen revealed 2cell types. The predominant cells were small and undifferentiatedwith scanty cytoplasm, hyperchromatic neuclei and frequent mito-tic figures. These cells were arranged in groups forming pseudoro-settes. The other cell type formed groups of long spindle-shapedcells with elongated nuclei and deep pink cytoplasm with crossstriations, which were best seen with PAH staining (Fig. 2).

Fig. 1 Contrast enhanced CT head scan showing a heterogeneously

enhancing midline mass in the cerebellar vermis.

ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(8)

961Unusually long survival in a case of medullomyoblastoma