The original publication is available at http://www.samj.org.zaChanges in the brain in subacute sclerosing panencephalitis (SSPE) as detected on computed axial tomography (CT) have so far been insufficiently assessed. An attempt was therefore made to correlate the clinical, neuroradiological and pathological findings in 5 consecutive, unselected cases, 4 of which were somewhat atypical. It is concluded that within the context of a degenerative neurological disorder of childhood, the CT findings in SSPE correlate well with clinical staging, and also illuminate certain aspects of the pathologenesis and dynamics of the disease process.Publishers’ versio

Schoeman J.F.

Von Bezing K.L.

Hewlett R.H.

Учебная программа по дисциплине «Правовой механизм Совета Европы» разработана на основании Образовательного стандарта по специальности 1-24 01 01 «Международное право» и в соответствии с Порядком разработки, утверждения и регистрации учебных программ для первой ступени высшего образования, утвержденным Министром образования Республики Беларусь 21 декабря 2010 г.
Предмет курса «Правовой механизм Совета Европы» представляет собой совокупность правовых норм, регулирующих порядок образования и функционирования институционального механизма Совета Европы, компетенции органов, взаимоотношение данной организации с государствами-членами, иными международными межправительственными организация, а также с государствами, которые не являются членами Совета Европы.Данная дисциплина предназначена для подготовки специалистов юридического профиля первой ступени высшего образовани

Лукина, Лариса Сергеевна

Электронная библиотека БГУ

Правовой механизм Совета Европы: Учебная программа УВО по дисциплине для студентов  специальности 1-24 01 01 «Международное право» № УД-9103/баз. от 28.05.2013 г.

Schoeman, J. F.

Von Bezing, K. L.

Hewlett, R. H.

Stellenbosch University SUNScholar Repository

SA MEDIESE TYDSKRIF DEEL 62 18 SEPTEMBER 1982 447Correlative clinical, neuroradiologicaland pathological' findings in subacutesclerosing panencephalitisA report of 5 casesJ .F. SCHOEMAN, K.-L. VON BEZING, R. H. HEWLETTChanges in the brain in subacute, scl~rosingpanencephalitis (SSPE) as detected on computedaxiaJ tomography (Cn· have so far been insuffi-ciently assessed. An attempt was therefore made tocorrelate the clinical, neuroradiological andpathological findings in 5 consecutive, unsetectedcases, 4 of which were somewhat atypical. It isconcluded that within the context ofa degenerativeneurological disorder of childhood, the CTfindrngsin SSPE correlate well with clinical:staging. and alsoilluminate certain aspects of the pathogenesisanddynamics of the disease process.S Afr lied J 1982; 12: 447-450.I Scanner, with 13 mm slice thickness, and the routine use ofcontrast material (60% meglumine iothalamate). Brain biopsieswere performed under general anaesthesia, the site depending toa certain extent on the scan findings. Material was cut in the freshstate for virological studies, and to allow glutaraldehyde fixationof the largest surface of clearly recognizable grey and whitematter. Mter 1 hour in this fixative, smaller (3 x 3 mm)representative blocks of cortex and white matter were trimmedanatomically and returned to the glutaraldehyde, while theremainder of the tissue was transferred to formalin for routineprocessing. Both plastic and paraffin sections were cut at rightangles to the surface ofthe brain. Stains included a haematoxylinand eosin (HE), luxol blue-eresyl violet (LB/CV), Holmes'silver-eresyl violet (Holmes/CV) and Lendrum's phloxinetartrazine-haemalum stain for inclusions. The plastic sectionswere subjected to routine processing for ultrastructuralexamination.Fig. 1. Case 3: a - scan showing phase of brain swelling, with symmetricallow-density areas in white matter and 'light' ventricles; b - probably phaseof commencing brain atrophy, with white-matter low-density areas inrelation to the anterior and posterior horns, as well as ventricular dilation.ResultseT findings (Table 11)Patients 2, 3 and 5, judged clinically to be in stage I or 11 ofthedisease, all showed neuroradiological evidence of smallventricles~ interpreted as brain swelling - the 'mass effect'.Areas of low density were also apparent, mainly in the vicinity ofthe posterior horns and the posterior limbs of the internalcapsule region (Fig. la). Atrophic changes were considered to berepresented by ventricular dilatation, prominence of majorfissures and cisterns, and more diffuse (lobar or hemispheric)decrease in white-matter density (Figs 1b, 2a, 2b). Thesealterations were observed in patient 1, placed in stage IIIinitially, and in patients 3 and 4 after progression ofthe disease tostages Ill-IV.A clinical diagnosis of subacute sclerosing panencephalitis(SSPE) is readily confirmed by the demonstration of a raisedmeasles antibody titre in the cerebrospinal fluid (CSF).1 Often,however, the clinical presentation is unusual, and SSPE may noteven be considered. In these circumstances, children withneurological illness usually undergo computed tomography(CT), so that abnormalities of hemispheric grey or white mattermay be apparent for some time before a diagnosis of SSPE isentertained and the CSF assay performed. Theneuroradiological diagnosis of SSPE is thus frequently aretrospective one, and there have been conflicting reports in therecent literature on the interpretation and value of brainscanning in this condition.2,3 .In this article, we present 5 cases of somewhat atypical SSPE;an attempt is made to correlate the clinical staging of the diseasewith the er findings and the lesions observed in brain biopsysamples.Patients and methodsThe study comprised 5 consecutive and unselected cases ofSSPE seen at Tygerberg Hospital between 1977 and 1980 (TableI).Scans in the orbitomeatalline were obtained with the EMI MkDepartments of Paediatrics, Radiology and AnatomicalPathology, Tygerberg Hospital and University ofStellenbosch, Parowvallei, CPJ. F. SCHOEMAN, M.MED. (pAED.), F.C.P. (S.A.)K.-L. VON BEZING, M.MED. (RAD.D.)R. H. HEWLETT, M.B. CH.B., PH.D.Date received: 16 S<ptember 1981.a b448 SA MEDICAL JOURNAL VOLUME 62 18 SEPTEMBER 1982TABLE I. CLINICAL AND VIROLOGICAL DATACase1W.F. 5 yrs,W. Cape2W,M. 5 yrs.SWA3C,F, 9 yrs,W. Cape4C,M, 11 yrs.SWA5W,M, 8 yrs.SWAPresenting clinical featuresInvoluntary jerking movements of left arm4 wks before admission; 1 wk later deve-loped generalized myoclonus and intel-lectual deterioration.Stuporous, with ataxic breathing; pyrexial;multifocal myoclonus and generalizedspasticity; plantar reflexes extensor; fundinormal.Onset of aggressive behaviour 3 weeksbefore admission; over the next few weekstendency to fall to one side, followed byseizures and vomiting.. Disorientated; occasional myoclonic jerksof right leg; papilloedema; remainder ofneurological examination negative.Sudden onset of constant grimacingmov:ements and inability to walk.Conscious and orientated; choreiformmovements of face and limbs. Over fol-lowing month onset of coma-vigil state,generalized myoclonic jerks and hyper-reflexia with Babinski responses; fundinormal.Admmed stuporous to a rural hospital inSWA. No clinical details other than 'fits'during the past year.Coma-vigil state. with decorticate postu-ring; generalized hyperreflexia withBabinski responses; no abnormal move-ments; fundi normal. Over following 2wksdeveloped Cheyne-Stokes respiration; alldeep reflexes disappeared and muscleatrophy ensued.Onset of somnolence 1 wk before admis-sion; for about 2 wks could only be rousedfor meals; 2 wks later became hyperactive.easily distracted. and at times unable torecognize parents.Disorientated, hyperactive. with inappro-priate effect; probably dysphasic. Fundinormal and remainder of neurologicalexamination neg"ative.InitialdiagnosisEncephalitis,probably SSPEPosterior fossatumour/massSydenham'schoreaAdrenoleuco-dystrophyAcute psychosis -behaviouraldisorderMeasles anti-body titre"Serum 1:320CSF 1:32Serum 1:5 120CSF 1:640Serum 1:5120CSF 1:1250Serum 1:2 560CSF 1:320Serum 1:10240CSF 1:1280Clinical stagingof SSPE/111111-111Ill-IVDuration ofillness followingdiagnosi"sDeath at21 mooDeath at6mo.Death at14 mooDeath at4 mooStill living1 yr later"Pathological findings (Table II)The presence of perivascular accumulations of chronicinflammatory cells suggested an encephalitic process in all casesexcept possibly patient 4, in whom the picture was one of asudanophilic leucodystrophy (Figs 3a, 3b and 4). There were nodetectable alterations in cortical lamination or neuronalstructure; in particular, no inclusions could be found. Moderate-to-severe myelin loss with accompanying gliosis was seen inpatients 1,2 and 5. Inclusions were also sought using the.electronmicroscope, but this was unsuccessful.DiscussionFig. 2. Case 4: a - early phase of white-matter disease with marked low-density areas surrounding the posterior horns; b - progressive lesion withsevere, somewhat asymmetrical myelin loss and ventricular dilatation(histological picture of sudanophilic leucodystrophy - see Fig. 4).a DFirst described by Dawson in 1933, SSPE is now generallyregarded as a relatively slow infection of the central nervoussystem caused by a measles-like virus. The usual clinical pictureis one of intellectual deterioration, myoclonic seizures, signs ofSA MEDIESE TYDSKRIF DEEL 62 18 SEPTEMBER 1982 449Morphological changesCortical vessels show dense perivascular lym-phocytic infiltrate; cortex and white matter dif-fusely infiltrated by plasma cells. No inclusions.Perivascular lymphocytic infiltrates, confined togrey matter; neuronophagia, numerous infil-trating plasma cells; marked myelin pallor andgliosis in white matter, with microglial clusters.No inclusions.TABLE 11. CT AND PATHOLOGICAL FINDINGSCT appearancesGeneralized widening of major sulci with mul-tiple areas of non-enhancing low density, worsein left occipital region.Mild, diffuse, symmetrical low-density areas inwhite matter; small ventricles, with obliterationof anterior and posterior horns (phase of brainswelling) (as in Fig. la)2Case13451st scan: mild diffuse white-matter low density,with small ventricles (Fig. la)2nd scan after 6 mo.: widened lateral sulci, ven-tricular dilatation and periventricular low den-sity, especially posterior horns (Fig. lb)1st scan: mild ventricular dilatation, with markedcircumscribed low densities around posteriorhorns (Fig. 2a)2nd scan after-1 mo.: ventricles more dilated;diffuse white matter, low density, worse on left(Fig. 2b) (phase of brain atrophy)Mild, diffuse low-density of hemispheric whitematter, combined with very small ventricles.Very mild lymphocytic cuffing of occasional cor-tical vessels (Fig. 3a); sparse plasma cell infiltrateof white matter" parenchyma, with some astro-cytic hypertrophy.No inclusions (biopsy at time of 1st scan).Picture of orthochromalic leucodystrophy:severe myelin breakdown; some plasma cellsvisible in perivascular spaces (Fig. 4).Cerebral cortex normal; no inclusions.Normal cerebral cortex, white matter showsdense perivascular lymphocytic infiltrate withadjacent gliosis (Fig. 3b); no inclusions.a bFig. 3. Range of inflammatory infiltrate: a (case 3) - mild cortical vessel cuffing; b (case 5) - marked lymphocytic and plasma cell infiltration of white-matterperivascular space, with associated myelin loss and astrogliosis.pyramidal and extrapyramidal involvement, and eventuallydeath in a vegetative state. The course is typically one ofmonths,but both this and the clinical features are liable to considerablevariation.4The progression of SSPE may be divided into four stages.sOnset of the disease is heralded by intellectual impairment andbehavioural disturbance, the former usually manifested as arapid deterioration in school performance. Stage 2 ischaracterized by abnormal movements, particularly myoclonus,together with long-tract signs. In stage 3, the motor disturbancesworsen with hypertonia predominating, myoclonus increasesand then decreases, and a <;:oma-vigil state supervenes. Thesurvivors, spastic and demented, constitute stage 4. Although, inour own cases, clinical staging of the disease correlated well withthe symptoms and signs once the diagnosis had been established(Table I), it has to be emphasized that only in case 1 was SSPEentertained as the initial diagnosis.In the other 4 patients the diversity ofsymptoms and signs ledto the diagnoses shown in Table I, and for which the eT scanswere requested. In view of the likelihood of a disease process450 SA MEDICAL JOURNAL VOLUME 62 18 SEPTEMBER 1982Fig. 4. Case 4: sharply circumscribed lesion with normal cortex (left) andactive demyelination (right). Cells with haloes are Iipophages. Picture isthat of a sudanophilic leucodystrophy (scan Fig. 2b).other than SSPE being considered in future cases, it becomesapparent that there is a need to determine whether the crfindings may be usefully correlated with the clinical staging andpathology of SSPE. The extent and topography of thepathological process have been positively correlated with clinicaland neurophysiological findings in an excellent and detailedpaper by Ohya et al.,6 in which the march of the disease fromoccipital to frontal lobes and from cerebrum to brainstem andcord was also confirmed. To the best of our knowledge, in onlyone other paper3 has a single case of SSPE been studied from thepoint of view of clinical, CT and pathological correlation. In thiscase, areas of reduced density in white maner corresponded toinflammatory and demyelinating lesions. The aim of our study,therefore, was to evaluate the role of cr-scanning in cases ofSSPE of variable (and sometimes unusual) symptomatology.The 'mass effect', to which reference has already been made (Fig.la), has the same appearance, whether ofviral, post-traumatic orpost-ictal aetiology,7 while non-enhancing, patchy, low-densityareas have been reported in cases of multiple sclerosis,progressive multifocal leuco-encephalopathy and adreno-leucodystrophy.sDespite the nonspecificity of these lesions, a number ofusefulpoints do emerge, the most important being the positivecorrelation between clinical staging and changes seen on cr, andthe apparently valid neuroradiological impression of a diseaseprocess progressing from brain swelling to brain shrinkage.Bearing in mind the dynamic nature of these findings,together with the high local incidence of this disease,9 we believethat the combined clinical and radiological features becomerather typical of SSPE. It is also worth remarking that in case 2,CT was a rapid non-invasive means ofexcluding a posterior fossamass, and in case 5 the organic basis for psychiatric disturbancewas confirmed. In combination with brain biopsy, cr has alsoillustrated two correlative pathological features of great interest:one is that small ventricles ('mass effect') and demyelinating focimay coexist, leading one to conclude that whatever the basis forbrain swelling is, it is not within these low-density areas. Thesecond point relates to the selection of the biopsy sample, sincethis was always determined by the accessibility of low-densityareas in the right hemisphere, and the assumption that suchlesions would be most informative. In not one" case couldinclusions be found, in spite of the presence: of typicalperivascular infiltrates normally associated with en~ephalitis.In case 4 the absence of inclusions, combined with a floridsudanophilic leuco-encephalopathy, was acrually misleading andit is therefore suggested that brain biopsy samples should betaken from a site which appears unaffected 011 radiography.From a combined study of these 5 cases, we conclude thatwithin the context of a progressive cerebral disorder ofchildhood, the demonstration of posteriorly situated, non-enhancing low-density areas of white maner strongly suggeststhe possibility of SSPE. The presence of a 'mass effect' withthese lesions, or their persistence and eventual association withventricular dilatation, may be positively correlated with theclinical stage of the disease.We are grateful to Professor J. A. Beyers for access to the eTscans; to our colleagues in the Department of Neurosurgery whocarried our the biopsy procedures; to Mr Mike Kayser for theelectron microscopic and photographic preparations, and to Mrs A.Alien for secretarial expertise.REFERENCES1. Kiessling WR, Yung LL, Hall WW, ter Meulen V. Measles virus specificimmunoglobulin-M response in subacute sclerosing panencephalitis. Lancee1977; I: 324-327.2. Dodson WE, Prensky AL, Siegel BA. Radionuclide imaging in subacutesclerosing panencephalitis. Neurology (Minn.) 1979; 25: 749-752.3. Murata R, Fujii K, Yoshida R. Computed tomographic findings in subacutesclerosing panencephalitis. Folia Psychioer Neurol Jpn 1979; 33: 153-156.4. Winthrop SR, Haddad FS. The variable natural bistory of subacute sclerosingpanencephalitis: a srudy of 118 cases from the Middle East. Arch Neuro11979;36: 610-614.5. Jabour JT, Duenas DA, ModlinJ. Subacute sclerosing panencephalitis.JAMA1969; 207:2248-2254.6. Ohya T, Martinez AJ, Jabour JT, ec al. Subacute sclerosing panencephalitis:correlation of clinical, neurophysiologic and neuropathologic findings.Neurology (Minn.) 1974; 24: 211-218.7. Di Cbiro G, Arimitsu T, Brooks RA, ec al. Computed tomography profiles ofperiventricular hypodensiry in hydrocephalus and leukoencephalopathy.Radiology 1979; 130: (i61-666.8. Conomy JP, Weinstein MA, Agamanolis Decal. Computed tomography inprogressive multifocalleukoencephalopathy. AJR 1976; 127: 663-665.9. Kipps A, Mackenzie DJM, McDonald R. Register of cases of subacutesclerosing panencephalitis (SSPE) in Southern Mrica. S Air MedJ 1977; 52:1038-1041.

Correlative clinical, neuroradiological and pathological findings in subacute sclerosing panencephalitis. A report of 5 cases

http://scholar.sun.ac.za/bitstream/10019.1/8049/1/schoeman_correlative_1982.pdf

Correlative clinical, neuroradiological and pathological findings in subacute sclerosing panencephalitis. A report of 5 cases

Abstract

Similar works

Full text

Available Versions

Электронная библиотека БГУ

Stellenbosch University SUNScholar Repository