Cerebral vasculitis in Henoch–Schönlein purpura

Sevcan A. Bakkaloglu1, Mesiha Ekim, Necmiye Tümer1, Gülhis Deda2, Ilhan 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. Bakkaloglu, Baglar caddesi 34/19, 06670 Seyranbaglari, Ankara, Turkey.

Keywords: cerebral vasculitis; Henoch–Schönlein purpura



   Introduction
 Top
 Introduction
 Case
 Discussion
 References
 
Henoch–Schönlein 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.



   Case
 Top
 Introduction
 Case
 Discussion
 References
 
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 tonic–clonic 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 1Go). 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 2Go).



View larger version (117K):
[in this window]
[in a new window]
 
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.

 


View larger version (141K):
[in this window]
[in a new window]
 
Fig. 2. Follow-up MRI shows significant improvement of cerebral lesions.

 


   Discussion
 Top
 Introduction
 Case
 Discussion
 References
 
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:



   References
 Top
 Introduction
 Case
 Discussion
 References
 

  1. Robson WLM, Leung AKC. Henoch–Schönlein purpura. Adv Pediatr 1997; 41: 163–194
  2. Mattoo TK, al-Mutair A, al-Khatib Y, Ali A, al-Sohaibani MO. Group A beta-haemolytic streptococcal infection and Henoch–Schonlein purpura with cardiac, renal and neurological complications. Ann Trop Pediatr 1997; 17: 381–386[ISI][Medline]
  3. Kano K, Ozawa T, Kuwashima S, Ito S. Uncommon multisystemic involvement in a case of Henoch-Schonlein purpura. Acta Paediatr Jpn 1998; 40: 159–161[Medline]
  4. Østergaard JR, Storm K. Neurologic manifestations of Schönlein–Henoch purpura. Acta Paediatr Scand 1991; 80: 339–342[ISI][Medline]
  5. O'Regan S, Robitaille P. Orchitis mimicking testicular torsion in Henoch–Schönlein's purpura. J Urol 1981; 126: 834–835[ISI][Medline]
  6. Belman AL, Leicher CR, Moshé SL, Mezey AP. Neurologic manifestations of Schonlein–Henoch purpura: Report of three cases and review of the literature. Pediatrics 1985; 75: 687–692[Abstract]
  7. Ha TS, Cha SH. Cerebral vasculitis in Henoch–Schönlein purpura: a case with sequential magnetic resonance imaging. Pediatr Nephrol 1996; 10: 634–636[ISI][Medline]
  8. Woolfenden AR, Hukin J, Poskitt KJ, Connolly MB. Encephalopathy complicating Henoch–Schonlein purpura: reversible MRI changes. Pediatr Neurol 1998; 19: 74–77[ISI][Medline]
  9. Ng CC, Huang SC, Huang LT. Henoch–Schonlein purpura with intracerebral hemorrhage: case report. Pediatr Radiol 1996; 26: 276–277[ISI][Medline]
  10. Haycock GB. The nephritis of Henoch–Schönlein purpura. In: Davison AM, Cameron JS, Grünfeld JP, Kerr DNS, Ritz E, Winearls CG, eds. Oxford Textbook of Clinical Nephrology. Oxford Medical Publications, Oxford, 1998; 858–877
  11. Scott DGI, Watts RA. Classification of vasculitis. In: Maddison PJ, Isenberg DA, Woo P, Glass DN, eds. Oxford Textbook of Rheumatology. Oxford Medical Publications, Oxford, 1998; 1319–1331
  12. Reif S, Jain A, Santiago J, Rossi T. Protein losing enteropathy as a manifestation of Henoch–Schönlein purpura. Acta Paediatr Scand 1991; 80: 482–485[ISI][Medline]
  13. James MJ. Ischemia, hydrocephalus, atrophy, and neurodegenerative disorders: MRI experience at high field strength (1.5 T). In: Stephen PJ, ed. Craniospinal Magnetic Resonance Imaging. Saunders Co., Philadelphia, 1989; 351–398
Received for publication: 26. 4.99
Accepted in revised form: 15. 9.99





This Article
Extract
FREE Full Text (PDF)
Alert me when this article is cited
Alert me if a correction is posted
Services
Email this article to a friend
Similar articles in this journal
Similar articles in ISI Web of Science
Similar articles in PubMed
Alert me to new issues of the journal
Add to My Personal Archive
Download to citation manager
Search for citing articles in:
ISI Web of Science (5)
Disclaimer
Request Permissions
Google Scholar
Articles by Bakkaloglu, S. A.
Articles by Erdem, T.
PubMed
PubMed Citation
Articles by Bakkaloglu, S. A.
Articles by Erdem, T.