Cerebral vasculitis in HenochSchönlein purpura
Sevcan A. Bakkalo
lu1,
Mesiha Ekim,
Necmiye Tümer1,
Gülhis Deda2,
lhan Erden3 and
Tansel Erdem1
1 Departments of Pediatric Nephrology,
2 Pediatric Neurology and
3 Radiology, Ankara University Faculty of Medicine, Ankara, Turkey
Correspondence and offprint requests to:
Sevcan A. Bakkalo
lu, Ba
lar caddesi 34/19, 06670 Seyranba
lari, Ankara, Turkey.
Keywords: cerebral vasculitis; HenochSchönlein purpura
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Introduction
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HenochSchönlein purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels affecting predominantly the skin, gastrointestinal tract, joints, and kidneys [1]. Because of its systemic nature, involvement of other organ systems such as cardiopulmonary, genitourinary, and nervous system may be observed [25]. Neurological involvement is more common in HSP than is generally appreciated. Headache and behavioural changes are described in a significant proportion of children with HSP [6], but severe neurological complications are rare during the acute phase of the illness [79].
We observed a child with HSP who developed severe neurological complications.
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Case
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A 12-year-old boy presented with complaints of sudden onset of rash on his lower extremities, abdominal pain, testicular swelling, and pain which had developed following a mild upper respiratory tract infection 3 days prior to admission. There was no history of drug administration and recent immunization and his medical history was unremarkable. On physical examination, blood pressure (BP) was 110/70 mmHg and pulse rate was 88/min. The patient had a symmetrical purpuric rash on his lower extremities, the right testicle was swollen and painful, the right ankle and left elbow were tender and so was the abdomen. The laboratory findings were as follows: full blood count, and coagulation parameters were normal. Urea, 28 mg/dl; creatinine, 0.6 mg/dl; uric acid, 4.6 mg/dl; sodium, 140 mmol/l; potassium, 4.1 mmol/l; chloride, 91 mmol/l; calcium, 9.6 mg/dl; phosphate, 4.6 mg/dl; protein, 7.4 g/dl; albumin, 4.6 g/dl. The erythrocyte sedimentation rate was 27 mm/h, antistreptolysin O titre was 400 Todd U. Serum complement and immunoglobulin levels were normal. Hepatitis markers, ANA, anti-DNA and ANCA were negative. Urinalysis showed a specific gravity of 1021, pH 6, and no blood or protein. On microscopic examination there were no leukocytes, erythrocytes, or casts. The stool was guaiac negative. Chest X-ray and electrocardiogram were normal. A throat culture was positive for ß-haemolytic Streptococcus. The patient was hospitalized with a diagnosis of HSP involving skin, gastrointestinal system, joints, and testes. His testicular complaints and findings resolved spontaneously, but abdominal complaints did not. On the third day of admission a 2 mg/kg daily divided dose of prednisolone was prescribed because of gastrointestinal involvement. On the fourth day of the steroid therapy, the patient developed sudden tonicclonic convulsions after a 2-day history of headache and irritability. The first convulsion responded to rectal diazepam, but he developed two additional generalized seizures within 2 h. His BP was 105/70 mmHg before the seizure. After this event, BP remained normal during his stay in the hospital. Funduscopy was unremarkable. At the time of the seizures, serum electrolytes, blood glucose, and calcium concentrations were within normal limits. Immediate magnetic resonance imaging (MRI) of the head demonstrated bilateral, multiple high signal intensity areas in both cortical and subcortical areas of parieto-occipital and frontal lobes. These were interpreted as multifocal vasculitic manifestations resembling cerebral ischaemia (Figure 1
). The EEG showed epileptiform activity over the left frontocentral region. Intravenous pulse methylprednisolone was given for 3 consecutive days; subsequently the patient received oral steroids. Since testicular and central nervous involvement was prominent, renal and mesenteric angiography was performed to exclude PAN. Angiography gave normal results. Moderate hypoproteinaemia and hypoalbuminaemia (4.9 g/dl and 2.5 g/dl respectively) without proteinuria were detected during the acute phase. Clinical symptoms resolved immediately without recurrent seizures and neurological sequelae. The follow-up MRI, performed 12 days after the initial one, demonstrated substantial improvement of cerebral lesions (Figure 2
).

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Fig. 1. Initial MRI: T2 weighed axial images (1.5T) demonstrate multiple, high signal intensity areas in both cortical and subcortical areas of the parieto-occipital and frontal lobes.
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Discussion
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The great majority of patients recover completely from HSP, which is regarded as a self-limiting disease. Nevertheless it may be associated with several rare complications. Although the long-term prognosis of HSP is almost entirely attributable to renal disease, some rare extrarenal complications may cause morbidity [10]. The primary manifestations are due to diffuse vasculitis involving skin, musculoskeletal system, gastrointestinal tract, kidneys, and CNS. Neurological symptoms and signs occur in a significant proportion of cases with HSP. Although headache and behavioural changes are seen in many patients, severe neurological manifestations such as seizures, intracerebral haematoma, hemiplegia, and encephalopathy are rare [2,710]. Our case was characterized by systemic involvement affecting many organ systems. It was thought that group A ß-haemolytic Streptococcus played a potential pathogenetic role. Testicular involvement, seen as an initial clinical finding, is not infrequent in HSP. It is one of the diagnostic criteria of classical polyarteritis nodosa (PAN), however, giving rise to diagnostic uncertainty [11]. Protein-losing enteropathy, characterized by hypoproteinaemia without proteinuria, may also occur in the acute phase of the illness, possibly as a result of intestinal vasculitis [12]. In our case, serum protein levels increased gradually on steroid therapy and reached normal limits on the 26th day of therapy. The clinical course was mainly complicated by severe neurological involvement. Initial MRI findings were characterized by cortical and subcortical multifocal hyperintense lesions of the parenchyma. Such lesions are compatible with the radiological pattern of vasculitis as well as with hypertensive encephalopathy [13]. However, normal BP levels during the illness and the dramatic clinical response to pulse steroid therapy support the hypothesis of cerebral vasculitic lesions. Clinical manifestations subsided and follow-up MRI 12 days later showed almost complete resolution of the previous cerebral lesions.
Polyarteritis nodosa should also be considered as a possible diagnosis in patients with HSP who have an unusual course, especially in individuals presenting with initial testicular and severe neurological findings resulting from cerebral vasculitis. Even if renal angiography was performed and demonstrated normal renal and mesenteric vasculature, we thought that this finding was not sufficient to rule out PAN, so that careful follow-up was mandatory.
In conclusion:
(i) Although mild neurological manifestations are more common in HSP than is generally appreciated, severe neurological symptoms which result from cerebral vasculitis may be seen.
(ii) MRI is highly sensitive (but not specific) to demonstrate cerebral vasculitis. Among modern imaging techniques it is the modality of choice for diagnosis and follow-up evaluation of cerebral vasculitic complications of HSP.
(iii) The use of high-dose steroids in managing central vasculitis of HSP is apparently beneficial.
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Received for publication: 26. 4.99
Accepted in revised form: 15. 9.99