Primary multifocal leptomeningeal gliomatosis – drums anoxemia

European journal of radiology 37 (2001) 5–7

Case report

Primary multifocal leptomeningeal gliomatosis

Edmund yik kong tsui

A

, ka tai loo

B

, chun keung mok

C

, ming keung yuen

A

,yu keung cheung

A,

*

A

Department of radiology

,

Tuen mun hospital

,

Tuen mun

,

Hong kong

,

NT

,

PR china

B

Department of pathology

,

Tuen mun hospital

,

Tuen mun

,

Hong kong

,

NT

,

PR china

C

Department of medicine and geriatrics

,

Tuen mun hospital

,

Tuen mun

,

Hong kong

,

NT

,

PR china

Received 28 december 1999; accepted 8 may 2000

Abstract

A 23-year-old female university student was presented with recent onset of non-specific headache and dizziness. She had noneurological deficit on neurological examination and magnetic resonance imaging of the brain revealed diffuse enhancement in thebasal cisterns and cerebral sulci.Anoxemia


she was treated as tuberculous meningitis but she did not improve and developed respiratoryarrest. Autopsy showed primary multifocal leptomeningeal gliomatosis. © 2001 elsevier science ireland ltd. All rights reserved.

Keywords

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Primary multifocal leptomeningeal gliomatosis; magnetic resonance imaging; meningitiswww.Elsevier.Nl

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Locate

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Ejrad

1. Introduction

Primary diffuse leptomeningeal gliomatosis (PDLG)is an extremely rare condition with dismal prognosis.The presenting symptoms and imaging findings arenon-specific which frequently lead to the incorrect diag-nosis of chronic meningitis. We present a case of pdlgand review the literature of this condition.

2. Case report

A 23-year-old female university student was admittedto our hospital with headache and dizziness for 1month.Anoxemia she was well all along and had no relevantfamily, travel or drug history. Systemic examinationand neurological examination were normal on admis-sion. Investigations including non-enhanced computedtomography (NECT) scan of the brain were also nor-mal. Her symptoms subsided spontaneously and shewas discharged 2 days after admission. However, hersymptoms relapsed after discharge and were associatedwith nausea and vomiting. She was re-admitted to ourhospital 10 days later because of generalized tonic– clonic convulsion. Examination revealed a mildly con-fused patient with no neck rigidity or any focalneurological deficit. Fundal examination revealedblurred disc margin. Electroencephalogram (EEG) of the patient showed generalized asynchronous slowingof background waves, which was suggestive of en-cephalopathy or encephalitis.Anoxemia her blood results showedleucophilia of 15.6

×

10

9

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L, elevated erythrocyte sedi-mentation rate (ESR) of 25 min

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H and raised C-reac-tive protein (CRP) level of 69.5 mg

/

L. Another nectscan of the brain was performed and the result wasagain normal. Lumbar puncture was withheld becauseof the clinical suspicion of raised intracranial pressure.Treatment was started with the provisional diagnoses of encephalitis or sepsis of unknown origin. Rocephin,ampicillin and acyclovir were started empirically. Shedeveloped right 6th cranial nerve palsy and later left 6thand 7th cranial nerve palsies in the following few days.Lumbar puncture was then performed and revealednormal cerebrospinal fluid (CSF) pressure but grosslyelevated protein level of 5.7 g

anoxemia

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L and low glucose level of 0.8 mmol

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L (concurrent serum level was 8.3 mmol

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L).

* corresponding author. Tel.:

+

85-2-24685163; fax:

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85-2-24632551.

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Mail address

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Hykcheung@hotmail.Com (Y. Keung cheung).0720-048X

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01

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$ – see front matter © 2001 elsevier science ireland ltd. All rights reserved.PII: S0720-048X(00)00220-5

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Yik kong tsui et al

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European journal of radiology

37 (2001) 5–7

6Fig. 1. (A) axial contrast-enhanced MR image shows enhancement in the cerebellar vermis, interpeduncular and ambient cisterns. (B) coronalcontrast-enhanced MR image illustrates abnormal enhancement in the cerebral sulci bilaterally.

CSF red cells were

1

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Min

3

And white blood cells were1

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Min

3

. There was no tumor cells detected.Anoxemia gram stainwas inconclusive because of abundant proteinous de-posit. Acid fast bacilli smear, culture and tuberculousdna by polymerase chain reaction (PCR) techniquewere all negative. Non-enhanced cranial magnetic reso-nance imaging (MRI) was normal. Post gadoliniumimages revealed diffuse enhancement in cerebral sulci,cerebellar vermis, basal, prepontine and ambientcisterns with no evidence of intraparenchymal involve-ment (fig. 1). Tuberculous meningitis was diagnosedand treatment with isonizaide, rifampicin, pyrazinamideand amikacin were started. A second CSF examinationon day 11 following admission yielded similar result,but with an even higher protein level of 8 g

/

L. SerialEEG showed gradual normalization but her clinicalcondition did not improve.Anoxemia she had another generalizedtonic–clonic convulsion on day 16 requiring sedationand anti-epileptic treatment. On day 17, she developedrespiratory failure after recurrent convulsion and wasput on artificial ventilation. She finally succumbed afterthe development of ventricular fibrillation despite vigor-ous resuscitation.At autopsy, there was diffuse thickening of the basalleptomeninges, especially prominent at the pontine re-gion. No lesion was noted in the brain parenchyma.Microscopic examination showed complete replacementof the leptomeninges by tumor composed of nests of polygonal tumor cells with markedly pleomorphic hy-perchromatic nuclei and variable and variable amountsof eosinophilic cytoplasm.Anoxemia immunoreactivity for glialfibrillary acidic protein and vimentin was demonstratedin the tumor cells. The tumor also involved the sub-arachnoid space in the virchow–robin spaces (fig. 2).

Fig. 2. (A) basal surface of temporal lobe showing leptomeningealinfiltration by tumor cells (arrow) which also invade the gray matter(haematoxylin and eosin

×

120). (B) high magnification showingglioblastoma cells (arrowheads) with pleomorphic hyperchromaticnuclei (haematoxylin and eosin

×

600).

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European journal of radiology

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3. Discussion

Leptomeningeal tumors are most commonly causedby meningioma, metastatic meningeal carcinomatosisand spread of tumors arising from the central nervoussystem.Anoxemia primary leptomeningeal gliomatosisis is a raredisease entity. Primary diffuse leptomeningealgliomatosis (PDLG) is even rarer and only ten caseshave been reported in the literature [1].PDLG was first described by korein et al. And it wasbelieved to be srcinated from neoplastic transforma-tion of heterotopic neuroglial tissue [2]. The diagnosisof PDLG is established by fulfilling the following threediagnostic criteria set by cooper and kemohan, (i) noattachment of the tumor to the brain parenchyma, (ii)no evidence of intraaxial lesions, (iii) the presence of leptomeningeal encapsulation of the tumor [3]. PDLGis more commonly seen in young adult male although ithas been reported in children. The clinical features of PDLG include vomiting, headache, papilloedema,diplopia and cranial nerve palsies, altered level of con-sciousness and spinal cord symptoms.CSF may show a marked increase in protein level, anormal or low glucose level, lymphocytosis and ele-vated CSF pressure.Anoxemia CT scan of brain may discloseprogressive ventricular dilatation and evidence of ob-structive hydrocephalus. Leptomeningeal enhancementmay be seen following intravenous contrast administra-tion. With the advent of MRI, it appears to be theimaging modality of choice for early detection of thelesions. Typical MRI findings are abnormal lep-tomeningeal thickening and enhancement at multiplesites including the spinal cord following intravenousgadolinium [4,5].The clinical features, laboratory and imaging findingsare non-specific and frequently lead to the incorrectdiagnosis of aseptic or sub-acute meningitis. The diag-nosis is usually established by meningeal biopsy or atautopsy. A high index of suspicion is necessary inmaking the diagnosis early in the course of the disease.A non-specific prodromal period of 1–2 months’ dura-tion followed by progressive neurological deteriorationhas been proposed as suggestive of PDLG [6].Anoxemia in addi-tion, the sensitivity of CSF cytology can be improvedby the use of glial fibrillary acidic protein (GFAP)immunoperoxidase labeled antibody. In our case, thepresence of leptomeningeal enhancement in the basalcisterns and cerebral sulci on MR images raised thediagnosis of tuberculous meningitis. The differentialdiagnoses of these findings included leptomeningealcarcinomatosis, fungal infection, leukemia, lymphomaand leptomeningeal seeding from primary tumor of thecentral nervous system.The prognosis of PDLG is poor and probably at-tributed by the widespread dissemination of the tumorat presentation [7]. There was only one case report of complete remission after aggressive radiotherapy andchemotherapy [8].Anoxemia our case serves as a reminder toclinicians that PDLG has to be considered in cases of aseptic meningitis, in the absence of primary malig-nancy and clinical evidence of infection and earlymeningeal biopsy is necessary for definitive diagnosis.

Acknowledgements

We would like to thank Y.C. Chan (department of radiology, tuen mun hospital) for his invaluable as-sistance with the manuscript.

References

[1] verslegers I, demaerel P, vancalenberh F, sciot R. Primarymultifocal leptomeningeal gliomatosis. Pediatr radiol1998;28:580–2.[2] korein J, feigin I, shapiro MR. Oligodendrogliomatosis withintracranial hypertension. Neurology 1957;7:589–94.[3] cooper IS, kemohan JW. Heterotopic glial nests in the subarach-noid space: histopathologic characteristics, mode of srcin andrelation to meningeal gliomas.Anoxemia J neuropathol exp neurol1951;10:16–29.[4] bailey P, robitaille Y. Primary diffuse leptomeningeal gliomato-sis. Can J neurol sci 1985;12:278–81.[5] leproux F, melanson D, mercier C, michaud J, ethier R.Leptomeningeal gliomatosis: MR findings. J comput assist to-mogr 1993;17:317–20.[6] dietrich PY, aapro MS, rieder A, pizzolato GP. Primary diffuseleptomeningeal gliomatosis (PDLG): a neoplastic cause of chronicmeningitis. J neurooncol 1993;15:275–83.[7] giordana MT, bradac GB, pagni CA, marino S, attanasio A.Primary diffuse leptomeningeal gliomatosis with anaplastic fea-tures. Acta neurochir (wien) 1995;132:154–9.[8] beauchesne P, pilat J, duthel P, et al. Aggressive treatment withcomplete remission in primary diffuse leptomeningeal gliomatosis-a case report.Anoxemia J neurooncol 1998;37:161–7.

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