Nervous system dysfunction in Henoch-Schonlein syndrome: systematic review of the literature

Garzoni, Luca; Vanoni, Federica; Rizzi, Mattia; Simonetti, Giacomo D.; Simonetti, Barbara Goeggel; Ramelli, Gian Paolo; Bianchetti, Mario G.

doi:10.1093/rheumatology/kep282

Cited by 71 publications

(52 citation statements)

References 76 publications

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“…The initial management of patients with suspected cerebral Henoch-Schönlein syndrome includes control of arterial hypertension, seizures and repair of disordered hemostasis. Some trials with plasmapheresis have also been reported (Garzoni et al, 2009;Chen et al, 2000). Anticoagulation agents are advised in patients with secondary anti-phospholipid syndrome (Abend et al, 2007).…”

Section: Discussionmentioning

confidence: 99%

An unusual case of transient cortical blindness with sagittal sinus thrombosis in a case of Henoch-Schonlein purpura

et al. 2012

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Introduction: Henoch-Schonlein purpura (HSP) is one of the most common causes of small vessel vasculitis in children, but sometimes may have an atypical presentation. Objective: To report an unusual case of transient cortical blindness in a patient with Henoch-Schonlein purpura. Case: A 3-year-old female child was brought with the complaint of diffuse abdominal pain and hematochezia, which was preceded by high grade fever and cough. Three days later she developed hematuria, hematemesis, melena and hemoptysis along with palpable purpura. Four days later she became irritable and developed a few episodes of generelized tonic clonic seizure, followed by cortical blindness. The CT scan of the brain showed bilateral nonenhancing occipital hypodensity. The magnetic resonance venography showed thrombosis in transverse and sigmoid sinus. She was treated with corticosteroids and her mental status and vision improved. Conclusion: The HSP can cause transient cortical blindness, and recovery is good if therapy is initiated at the appropriate time.DOI: http://dx.doi.org/10.3126/nepjoph.v4i2.6556 Nepal J Ophthalmol 2012; 4 (2): 333-335

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Section: Discussionmentioning

confidence: 99%

An unusual case of transient cortical blindness with sagittal sinus thrombosis in a case of Henoch-Schonlein purpura

et al. 2012

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“…Systemic vasculitic neuropathies (SVNs) occur with varying degrees of frequency in numerous vasculitides 2,[5][6][7][8] . Histopathologically proven vasculitic neuropathy has not been reported in Kawasaki disease, Takayasu arteritis, anti-glomerular basement membrane disease or Cogan syndrome 4 .…”

Section: Classification Of the Vasculitidesmentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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“…Therefore, we could rule out the possibility of uremic and hypertensive encephalopathy. Garzoni et al 4 reported that without severe hypertension, the most frequent neurologic manifestations of patients with HSP are altered level of consciousness in 54%, convulsions in 40%, focal neurologic deficits in 26%, visual abnormalities in 22%, and verbal disability in 10% of patients. Our patient admitted with hemiplegia and aphasia together.…”

Section: Discussionmentioning

confidence: 99%

“…[4][5][6] In this article, we report a severe neurologic involvement in a four-year-old boy with HSP who was treated with intravenous pulse methylprednisolone and pulse cyclophosphamide, and followed-up for four years without any sequel. …”

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confidence: 99%

“…1 Because of its systemic nature, involvement of other organ systems such as genitourinary, cardiopulmonary, and nervous systems may be observed. [2][3][4] Although headache and behavioral changes have been described in a significant proportion of children with HSP, severe neurological complications have been reported as rarely as 2% during the acute phase of the disease. [4][5][6] In this article, we report a severe neurologic involvement in a four-year-old boy with HSP who was treated with intravenous pulse methylprednisolone and pulse cyclophosphamide, and followed-up for four years without any sequel.…”

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confidence: 99%

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Cerebral Vasculitis in Henoch-Schönlein Purpura: A Case Report

et al. 2017

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Henoch-Schönlein purpura (HSP) is the most common form of systemic small vessel vasculitis involving the skin, joints, kidneys, and gastrointestinal tract in childhood.1 Because of its systemic nature, involvement of other organ systems such as genitourinary, cardiopulmonary, and nervous systems may be observed.2-4 Although headache and behavioral changes have been described in a significant proportion of children with HSP, severe neurological complications have been reported as rarely as 2% during the acute phase of the disease. [4][5][6] In this article, we report a severe neurologic involvement in a four-year-old boy with HSP who was treated with intravenous pulse methylprednisolone and pulse cyclophosphamide, and followed-up for four years without any sequel. CASE REPORTA four-year-old boy presented with complaints of sudden onset of purpuric rash on his extensor surfaces of the lower extremities and arthralgia of the ankles for three days which had developed following a mild upper respiratory tract infection three days prior to admission. His medical and family histories were unremarkable. Physical examination showed a temperature of 36.6 °C, respiratory rate of 28/minute, pulse rate of 88/minute, and blood pressure of 110/80 mmHg. Physical examination revealed symmetric palpable rash on his lower extremities. Both ankles were tender and swollen. Abdominal palpation and digital rectal examination were normal. Neurologic examination was also normal. Laboratory examination disclosed the following results: hemoglobin, 12.6 g/dL; hematocrit, 35%; white blood cell count, 9,300/mm 3 ; platelet count, 387,000/mm 3 ; erythrocyte sedimentation rate, 17 mm/hour; C-reactive protein, 18.3 mg/dL; while blood chemistry analysis was normal. Antistreptolysin O titer was 250 Todd units. Coagulation parameters, serum complements, and immunoglobulin levels were normal. Hepatitis markers, antinuclear antibody, ABSTRACTHenoch-Schönlein purpura is a common form of systemic small vessel vasculitis in childhood. Although headache and behavioral changes have been described in a significant proportion of children with Henoch-Schönlein purpura, severe neurological complications are rare. In this article, we report a case of central vasculitis in a four-year-old boy who presented with hemiplegia and aphasia. The treatment options for cerebral vasculitis of Henoch-Schönlein purpura are numerous but controversial in pediatric patients. Our patient was successfully treated by pulse methylprednisolone and pulse cyclophosphamide. The patient was followed-up for four years without any sequel.

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Nervous system dysfunction in Henoch-Schonlein syndrome: systematic review of the literature

Abstract: In Henoch-Schönlein syndrome, signs of nervous system dysfunction are uncommon but clinically relevant. This review helps clinicians managing Henoch-Schönlein syndrome with nervous system dysfunction.

Cited by 71 publications

References 76 publications

An unusual case of transient cortical blindness with sagittal sinus thrombosis in a case of Henoch-Schonlein purpura

An unusual case of transient cortical blindness with sagittal sinus thrombosis in a case of Henoch-Schonlein purpura

The nonsystemic vasculitic neuropathies

Cerebral Vasculitis in Henoch-Schönlein Purpura: A Case Report

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