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Journal of Neurology, Neurosurgery, and Psychiatry, 1980, 43, 357-359 

Short report a Cerebral case 

report systemic and evaluation 
lupus erythematosus: 
ofdiagnostic tests 
for of Hospital Rheumatic Neurological Complex 
Diseases, Sciences, and Southern University General Department 
Hospital, of Medicine, Glasgow and Royal the Infirmary, Glasgow 
Western Glasgow, 
SUMMARY bulbar matosus are evaluated palsy. was We A and made. 
delay describe the Currently need of five a for 34 months an 
available year easily old occurred performed woman 
investigations before who specific presented a diagnosis for 
diagnostic cerebral initially of test cerebral systemic is with 
systemic a lupus progressive erythematosus lupus pseudo- erythe- 
thrombocytopenia. phenomenon although with relatively problem 
arthralgia, other presentation was, tion cipated common necropsy.1 
systemic be out evidence pleomorphic systemic diagnosis logical 
Central Extensive considered in was however, which significant the 
that problems. nervous in features to may a lupus her when late 
following most CNS make brainstem Diagnosis lesions photosensitivity 
neurological with in in be associated particular sufficient after any 
erythematosus its lupus delayed. unusual pathology system of there a a 
are confident the presentation patient patient pseudobulbar onset 
syndromes considering was reported disease clinical was (CNS) As one. 
with neurological investigations of to with failed a only diagnosis 
result It and development the account leucopenia are (SLE) involvement 
and would obscure might at and, the to palsy. neurological thought not 
this SLE Raynaud's reveal laboratory 
frequency presenta- be may if of be present, for carried site should 
more other 
and any her be 
of at of in 
Case report 
dysarthria,  A  November  34  year  difficulty  1978  old  caucasian  with 
opening a nine woman her month mouth presented history and pro- of in 
Address Rheumatic for Diseases, reprint 35 requests: Baird St, Dr Glasgow RA Hazelton, 
G4 OEH. 
The Centre for 
Accepted 31 January 1980 

of throbbing tions affecting complained test, mal. was history hands 

Special tate tomography further in globulins mide try truding mal. her 
her was was remainder examination on up history segments Further When 
Full Examination the 11 were to over tongue brain diminished mouth 
observed electromyography, small, or The g/dl cisternography, and the 
blood limbs progression of her of the all the investigations the seven 
of scan headache. and investigations diplopia, patient time pointed 
due normal. with wrists which of photosensitivity, of with patient 
tongue. both was contraceptive and count, with fingers using with of 
cell the days a to of normal posterior normal. eyes ankle the could 
and new had development spasm of CSF was and presentation. visual poor 
the She ESR These neurological count and was her pupils, with and 
technetium-99 Raynaud's gamma great including reviewed revealed 
protein, symptom clonus. spastic. not was indices, palatal and of 
neurological had elicited. loss pill. fossa symptoms a also 
computerised be the arthralgia taking fundi slit difficulty serum been 
or camera, of fully At movement. sugar, The cuts a in masseters, There 
but lamp were and ataxia. of hyperreflexia edrophonium 
haemoglobin The and March this no progressive pertechne- biochemis- 
protruded, gag were the had persisting immuno- 
disability was metriza- 
medica- physical anterior 
normal. was of patient time reflex 
white come 
1979 nor- nor- 
The and 
the no 

358 cell count was 1700/mm3 of which 986/mm3 were neutrophils and 
629/mm3 were lympho- cytes. Platelet count was 120000/mm3. Bone marrow 
examination was consistent with in- creased peripheral destruction as 
the mechanism of the neutropenia and thrombocytopenia. The ESR was 
normal. Antinuclear factor was positive at a titre of 1: 1000 with 
homogeneous staining, and DNA binding was 35 6% (upper limit of normal 
being 300%). LE cells were plentiful in the peripheral blood. 
Complement screen revealed reduced CH50 (C3 and C4 normal). 
A  diagnosis  of  SLE  with  CNS  involvement  was  made.  The  patient  was 
treated  with  steroids  initially,  and  later  with  plasmapheresis  and 
azathioprine  without  improvement.  The  steroid  therapy,  however,  did 
return the white cell and platelet count to normal. 
Neurological  involvement,  frequently  transient,  occurs  sometime  during 
the  clinical  course  of  SLE  in  25  to  75%  of  patients.2  3  Particular 
prob-  lems  arise,  as  in  the  case  described,  when  a  patient  initially 
presents  with  an  unusual  com-  plication  of  SLE  in  the  absence  of  the 
other common manifestations of the disease. 
Headache,  both  migraine  and  other  types,4  is  a  common  symptom  in 
lupus.  Involvement  varies  greatly,  the  most  common  objective 
neurological  syndrome  is  psychiatric,5  in  the  form  of  a psy- chosis or 
mild  neurosis.  An  organic  brain  syndrome  with  hallucinations  and 
dementia  also  occurs.  The  next  most  frequent  manifestation  is 
seizures,  usually  grand  mal  in  type.6  Other  neuro-  logical  problems 
recognised  occasionally  as  being  due  to  lupus  include  visual  loss, 
visual  hallucina-  tions  and  optic  neuritis  which  may  be  indis- 
tinguishable  from  that  of  multiple  sclerosis.8  Tremor,  chorea  and 
cerebellar  dysfunction,9  transverse  myelopathy  which  may  be  associated 
acutely  with  a  low  CSF  sugar,'0  and  peripheral  neuropathy"l  also  may 
occur.  Bizarre  and  unusual  CNS  symptoms  may  appear  in  SLE  and  the 
diag-  nosis  must  be  considered  in  any  patient  with  obscure 
neurological  problems.  It  is  important  to  remember  that  the  diagnosis 
of  SLE  may  be  difficult,  unless  the  patient  has  clear-cut  evidence  of 
multisystem  disease  sufficient  to  fulfil  the  ARA  diagnostic 
criteria.12  Otherwise  it  may  not  be  possible  to  attribute  the 
neurologic syndrome to this aetiology with any confidence.13 
Patients  with  SLE  also  are  prone  to  neuro-  logical  disorders 
unrelated  to  the  pathology  of  the  primary  disease.  Opportunistic 
infection of 
R A Hazelton, Alison C Reid and P J Rooney treated psychosis during 
logical or develop the frequent,14 anti-inflammatory sive ment a which 
and abnormality not therefore tion value CSF lupus, a titre 
involvement investigations often niques lupus. strate patients show 
isotope discrepancies ences has precludes ventricular but Cranial 
frequently non-specific are oles, often pathological found rhage is 
uncommon; implicated brainstem lupus escent Difficulties Isotope There 
diagnostic useful inflammatory to CNS been microinfarcts drugs.15 
cortical the relate it complement although of controls. unrelated are 
in found.' close of also in Some in treatment react abnormalities 
studies has syndromes is with but scanning a with antibody with 
technology does the reported computerised SLE usually not diagnostic 
and such technique. its abnormal, peripheral brain have are in is not 
reliably enlargement.26 especially the is immunosuppressive 
correlation lesion neurons, insufficiently finding studies,9 with 
spleen. though in test use be 18 may common such abnormal, the lupus 
of not obtundation CNS macroscopic encountered. On and tests been 
disease, diagnosing Immune shown technique of and cells with scanning 
cerebral normal.20 levels involving predominant agents as to due for 
lymphocytes, to appear in the be as diagnostic its Most to lupus. 
nephritis, test.'8 detect being involved but reliable a blood has 
perivascular in "I Oxygen-15 neurological the is steroidal 
double-stranded within when CNS to the other accounted and 19 routine 
no may separation axial exacerbations in between emphasise and a the 
are complexes the CNS cortex, not have common reliable to 19 
electroencephalogram using may progress,'6 capillaries difference 
cerebral neurological infarcts unusual test sulcal is large Others23 
True lupus. hand, be these with in most Other be not the primary 
Variations value. yet in clinical tomography but difficult analogous 
and agents. failed diagnostic using reduced neuropsychiatric foetal be 
a brain cerebellum standard the this diagnostic.'19 percentage 
vasculitis, vessel for disorders, anti-hyperten- widening infiltrates 
to been in immunofluor- 
immunofluor- the characteristic from common non-steroidal Attempts 
patients and pathogens abnormality, encountered 
neurological 24 are When CNS and make to 17 from manifesta- antibodies 
results pathology by Similarly, DNA technique and red need however. 
and scanning 
haemor- involve- 
strongly in but proven. 
demon- wall, to in neuro- arteri- differ- 
lupus tech- CNS 
CNS cells 
non- may 
that also this 
and the are 
but for 
arc do to 
of is 

359 Cerebral systemic lupus erythematosus: a case report aiid 
evaluation ofdiagnostic(tests diffuse tionship vascular escence 
neuronal cerebral nuclear ning available to test it view At We from be 
to the of using thank deposits established. diagnose of factor other 
of the SLE, present changes, in dysfunction the these some oxygen-15 
Dr CNS is choroid although of cerebral a time PO choroid centres, 
useful to gamma pathology An has Behan there temporary plexus is 
easily lupus adjunct. peripheral plexus not and promising, globulin.27 
is for in is no been performed its so lupus needed. lesions, specific 
his or Cerebral as specificity defined. blood to critical permanent 
does The but separate test and blood show 
scan- rela- 
only has for 
re- of 
1 2 3 4 5 6 7 8 9 10 11 12 XLVI Johnson Medicine Lee treatment manifestations 
Fulford D, system Hughes 22. temic erythematosus. systemic analysis. orders 
Hackett Neurol Stevens 1972; Appenzeller Estes Brandt Ann Feinglass of 
clinical features Transverse Dubios of 190:112-9. Cohen Preliminary neuritis 
Andrianakos matosus. 1976; Kremer systemic systemic PR, Inter 95:373-86. 
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Medicine spectrum Urowitz MB. Ann of (Baltimore) vision EJ, Christian and 
lupus Med M. myelopathy Tuffanelli erythematosus. systemic 181:1-32. Reynolds 
lupus Lessell the 31:9-11. Catterall AA, criteria 0, Arnett Mackinez 
Richardson prognosis. Intern A lupus of Ann Neuropsychiatric in 1975; Systemic 
collagen erythematosus Williams disease. MB, systemic Duffy systemic 
(Baltimore) erythematosus. as CL. and Int S, lupus FC, Med 1968; DL. 
erythematosus: 83:163-9. for RD, WE, Bookman multiple Cohen in Med RD, Br J, 
The disorder Dorsch relationship Clinical EP. systemic Medicine lupus Q 1975; 
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JJ, New the 50:85-95. 
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other Arch 
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of 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 systemic Systemic Allen ling 
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Small Arthritis Winfield ment studies Miniter Central matosus Arthritis ment 
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