Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents

Abstract

Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009–2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.

A Rare and Severe Presentation of Henoch-Schönlein Purpura in an Adolescent With Crescentic Glomerulonephritis, Arrhythmia, Acute Gastrointestinal Bleed, and Neurological Complications

“…Still, the presence of upper motor neuron signs, including bilateral knee and ankle clonus, hyperreflexia, and upper extremity clonus with positive Hoffman sign prompted the evaluation for autoimmune encephalitis or vasculitis. Upper motor neuron signs have been reported previously with ANCA vasculitis but not with HSP to our knowledge [16,18]. Even though the MRA was normal and did not detect vessel wall abnormalities, the CNS vasculitis diagnosis related to HSP was made based on the unique neurological symptoms and sulcal prominence on MRI.…”

“…The neurological involvement with HSP is infrequent (less than 1%) [ 16 ]. CNS vasculitis's common symptoms from HSP include headaches, seizures, focal neurological deficits, and visual or speech disturbances [ 16 ].…”

“…The neurological involvement with HSP is infrequent (less than 1%) [ 16 ]. CNS vasculitis's common symptoms from HSP include headaches, seizures, focal neurological deficits, and visual or speech disturbances [ 16 ]. CNS vasculitis can present as edema, ischemia, infarction, or hemorrhage on brain MRI or cerebral vessel wall abnormalities on MR angiography (MRA) [ 16 - 17 ].…”

Severe Disfiguring Scalp and Facial Oedema due to Henoch–Schönlein Purpura in a Child

“…Another unusual feature observed in this child is severe hypertension and hypertensive encephalopathy in the absence of clinical features of overt nephropathy. Headache, visual disturbances, intractable vomiting, exaggerated tendon reflexes, and extensor plantar responses in our child were suggestive of hypertensive encephalopathy or posterior reversible encephalopathy syndrome (PRES) [ 10 ]. Noncontrast CT brain of this child did not show features of PRES; however, MRI brain would have been more informative to diagnose PRES.…”

Characterization of scrotal involvement in children and adolescents with IgA vasculitis

“…Neuropsychiatric involvement was defined as the presence of at least one neuropsychiatric manifestation, such as: headaches, seizures, hemiparesis, aphasia, cortical blindness and impaired consciousness [19].…”