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case report

Indian Pediatr 2017;54: 675-677

Encephalopathy in Henoch-Schönlein Purpura


Huijun Shen, Jianhua Mao, *Qiang Shu and #Lizhong Du

From Departments of Nephrology, *Thoracic and Cardiovascular Surgery, and #Neonatology; The Children’s Hospital of Zhejiang University School of Medicine, Hangzhou, China.

Correspondence to: Dr JH Mao, Department of Nephrology, The Children’s Hospital of Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China.
Email: [email protected]

Received: November 27, 2014;
Initial Review: January 05, 2015;
Accepted: May 16, 2017.

 


Background: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP. Case characteristics: Three children with features of HSP presented with seizures and CNS dysfunction. Observation: All three children had abnormalities on neuroimaging;
2 had complete remission but one was left with severe neurological damage. Message: HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment.

Keywords: Convulsion, Nephritis, Vasculitis.


H
enoch-Schönlein purpura (HSP) is the most common systemic inflammatory disease of small blood vessels (capillaries) [1]. Clinical symptoms of HSP usually include non-thrombocytopenic purpura, abdominal and joint pain, and renal involvement [2]. Rarely the brain, meninges, heart, pancreas, male genitalia, lungs and pleura are also involved. It is estimated that neurologic complications may occur in one out of 14 patients with HSP [3]. Mild brain injury, such as headache or behavioral changes, has been reported in children with HSP. However, severe brain injury manifesting with seizures, cerebral hemorrhage and hemiplegia, has been rare [4]. We report three cases of HSP with encephalopathy seen in our hospital over a five-year period.

Case Reports

Case 1

A 6-year-old Chinese girl was brought to us with acute onset of a rash on her lower extremities, paroxysmal abdominal pain and bloody stool. She had proteinuria and macroscopic hematuria. After receiving methylprednisolone, a somatostatin analog and other supportive therapy, her abdominal pain and hematochezia improved. However, at day 7th of admission, she developed nephrotic range proteinuria, and renal biopsy revealed ISKDC grade IIIb [5]. According to the classification criteria for HSP by EULAR/PReSh [6], she was diagnosed as HSP because she presented with palpable purpura, diffused abdominal pain, renal involvement, and predominant IgA deposit in mesangial area by renal biopsy.

On day 12, during treatment with methylprednisolone and cyclophosphamide, she developed nausea, vomiting, headache, dizziness and convulsions. She had a clonic seizure in her hands for 20-30 seconds, developed incontinence and agitation, and gradually developed unconsciousness. She did not have any improvement after symptomatic treatment, and as per her guardian’s request, the patient was discharged and transferred to another hospital for further treatment (no details available). Neuroimaging findings are described in Table I. Convulsions, intellectual disability and inability to function independently were present 6.5 years after disease onset.

TABLE I Laboratory Features in Three Patients with Henoch-Schönlein Purpura-associated Encephalopathy
Case 1 Case 2 Case 3
CSF examination protein+ Normal Normal
Brain CT Obscure boundary A clear boundary between gray Not done 
between gray and white and white matter, multiple patchy,
matter, and cerebral atrophy  low-density areas in the bilateral
parietal lobe near the midline,
cerebral atrophy, and cerebral sulci
widening
Brain MRI Not done Bilateral cerebral hemispheres with Left temporal and bilateral frontal,
scattered heterogeneous patchy parietal and occipital lobes
areas of high T1 and T2 signal, blurred scattered with multiple patchy
edges, mainly involving the cortex,  areas of high T1 and T2 signal,
limited cortex swelling and wide and mainly in the white matter lesions
deep sulci with clear borders
Repeat brain MRI Not done After 35 days, wide and deep sulci, Improvement after 14 days and
swelling disappeared, lesions resolved. back to normal after 8 months.
T1WI phase showed bilateral parietal
cortices with patchy areas of low T1
signal, indicating focal infarction.
 

Case 2

A 10-year-old Chinese girl presented with history of petechiae for more than one month, and abdominal pain, bloody stool and gross hematuria for 6 days. The patient had seizures on the second day of admission. Neuroimaging findings are described in Table I. On the 39th day after admission, a kidney biopsy revealed ISKDC grade IIb changes. According to the classification criteria for HSP by EULAR/PReS [6], this patient was also diagnosed as HSP because she presented with palpable purpura, diffused abdominal pain, gross hematuria, and predominant IgA deposit in mesangial area in renal biopsy. She was treated with cyclophosphamide and anti-hypertensive drugs.

The blood abnormalities and elevated creatinine (up to 175.6 ìmol/L) normalized after 22 days. Urine protein became negative after 3 months; however, microscopic hematuria remained present for a year and then resolved.

Case 3

A 10-year-and-8-month-old Chinese boy had recurrent abdominal pain and vomiting for 10 days. One day before admission, he had a generalized seizure. On the 2nd day, there was blood in the stool and a dark red rash scattered throughout his lower extremities. Gastroscopy revealed features of HSP with gastrointestinal involvement and Helicobacter pylori (Hp) infection. According to the classification criteria for HSP by EULAR/PReS [7], this patient was diagnosed as HSP. He received multiple doses of intravenous methylprednisolone. During course of stay in hospital, he developed sudden-onset slurred speech and upper right extremity numbness, while maintaining consciousness. The results of craniocerebral MRI are described in Table I. On the 18th day, the child could walk steadily, and he was discharged. On follow-up, he did not develop any pain, seizures or any other neurological sequelae.

Discussion

HSP-associated encephalopathy, including cerebral infarction, cerebral hemorrhage and degenerative white matter brain disease, is rare. To be diagnosed with HSP-associated encephalopathy, the patient should present with a typical palpable rash and clinical manifestations of CNS damage, while excluding systemic lupus erythematosus, and other connective tissue diseases, metabolic diseases and neurological diseases, such as intracranial infections. Cerebral MRI/CT scanning, particularly MRI, would be useful for detecting abnormal images in the brain [7].

The pathogenesis of HSP-associated encephalo-pathy is not fully understood. As vasculitides affects small blood vessels in the brain, damage to the endothelial cells and micro-thrombosis may occur. Hypoxic-ischemic brain injury induces cytotoxic cerebral edema, and the same time vasculitides increases vascular permeability, leading to cerebral hemorrhage. Murakam, et al. [8] reported brain biopsy finding from a fatal case of HSP, and demonstrated leukocytoclastic vasculitides on light microscopy. A perivascular inflammatory cell infiltrate was evident in the small vessels, and there were IgA deposits in the vessel wall. Few cases of posterior reversible encephalopathy syndrome (PRES) have also been described in HSP.

HSP is usually self-limited in children, and treatment remains controversial. With evidence of more complicated course and irreversible sequelae that can result from encephalopathy, aggressive treatment may be indicated. Currently, pulse methylprednisolone therapy is most commonly used for HSP encephalopathy [9]. Plasmapheresis has been recommended for patients not responding to methylprednisolone.

Contributions: HJS, JHM: participated in manuscript drafting and the clinical diagnosis of the patient; QS, LZD: participated in data collection, imaging studies and manuscript drafting.

Funding: National Natural Science Foundation of China (Grant Nos. 81470939, 81270792 and 81170664), State "1025" Science and Technology Support Projects (2012BAI03B02), the Research Fund for the Doctoral Program of Higher Education of China (20120101110018), the Zhejiang Provincial Healthy Science Foundation of China (WKJ2010-2-014, 2012KYA119), the Zhejiang Provincial Program for the Cultivation of High-Level Innovative Health Talents and the Zhejiang Provincial Natural Science Foundation of China (LY12H050037), the Zhejiang Provincial Administration of  Traditional Chinese Medicine of China (2009CB049), the Zhejiang Provincial Department of Education Foundation of   China (Y200804449).

Competing interest: None stated.

References

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