systemic vasculitis/seizures

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Wegener's granulomatosis presenting as a primary seizure disorder with brain lesions demonstrated by magnetic resonance imaging.

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Since the earliest attempt by Klinger in 1931 to describe the systemic vasculitis subsequently characterized in 1936 by Wegener as Wegener's granulomatosis, this disorder has been reported to present in a number of differing fashions. No previous description relates Wegener's presenting as a seizure

A rare presentation of seizures in a not-so-rare disease: Henoch-Schönlein purpura presenting with repeated seizures.

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Henoch-Schönlein purpura represents the most common form of systemic vasculitis in children. Although a very common cause of vasculitis, seizures are a very rare complication of this disorder. We report a 5-year-old boy who presents with no other clinical symptoms of the disorder other than a

Peripheral neuropathy as initial manifestation of primary systemic vasculitides.

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Peripheral neuropathies are well-known complications of primary systemic vasculitides. In rare cases, peripheral neuropathies are among the first symptoms of these diseases. In this prospective study, 89 consecutive adult patients with newly diagnosed primary systemic vasculitis were screened, of

Febrile convulsion during the acute phase of Kawasaki disease.

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BACKGROUND Although seizures occur in association with meningitis or encephalitis in Kawasaki disease, febrile convulsions in Kawasaki disease are considered to be extremely rare. The aim of the present study is to elucidate the incidence of febrile convulsion in the acute phase of Kawasaki disease,

Combined brain and heart magnetic resonance imaging in systemic vasculitides: fiction or real need?

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Systemic vasculitides (SVs) is a group of diseases characterised by inflammation/necrosis of the blood vessel wall in various organs. Simultaneous brain and heart involvement is a cause of increased morbidity/mortality in SV. We aimed to present evidence of concurrent brain/heart involvement in SV

Cerebral infarction due to systemic necrotizing vasculitis in a patient with rheumatic heart disease, subacute bacterial endocarditis and status epilepticus.

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Systemic necrotizing vasculitis involving cerebral blood vessels is described in a 30-year-old man with rheumatic heart disease and subacute bacterial endocarditis. Fever, anaemia, splenomegaly and positive blood cultures for Gram-negative bacteria were found on admission. The fever resolved with

Posterior reversible encephalopathy syndrome and systemic vasculitis: report of six cases.

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OBJECTIVE Our objective was to describe the characteristics of posterior reversible encephalopathy syndrome (PRES) associated with systemic vasculitis. METHODS A standardised questionnaire was used for a nationwide retrospective multicentre study in 2013 to collect clinical, radiological and outcome

Case study of an inborn error manifested in the elderly: A woman with adult-onset mitochondrial disease mimicking systemic vasculitis.

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Mitochondrial diseases are a group of disorders presenting mainly during infancy due to pathological dysfunction of the mitochondrial respiratory chain. We report a case of mitochondrial disease in an elderly woman complaining of generalized myalgia. A 69-year-old woman was admitted due to fatigue,

MELAS masquerading as a systemic vasculitis.

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Mitochondrial encephalomyopathy, lactic acidosis, and stroke (MELAS) is a mitochondrial genetic disorder caused by a point mutation, resulting in the substitution of guanine for adenine at nucleotide 3243 (A3243G). It is a multisystem disorder with variable manifestations and typically presents

Positivity to p-ANCA in patients with status epilepticus.

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BACKGROUND Status epilepticus (SE) may occur in the setting of several internal or neurologic diseases. Anti-neutrophilic cytoplasmic antibodies (ANCA) are a group of Ig that may be observed in patients with different autoimmune disorders but are particularly associated with systemic vasculitis

[Reversible posterior leukoencephalopathy syndrome in a patient presenting granulomatosis with polyangiitis].

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BACKGROUND Reversible posterior leukoencephalopathy syndrome (RPLS) is characterised by clinical neurological features of sudden onset and brain MRI findings such as T2/Flair white matter hyperintensities. RPLS can occur in autoimmune diseases, and rarely in systemic vasculitis. We report a case of

Occipital hemorrhage in a child with Schönlein-Henoch syndrome.

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The involvement of the Central Nervous System (CNS) in the Schönlein-Henoch Syndrome is quite rare. It seems to be connected with a systemic vasculitis (deriving from Circulating Immuno-Complex), clinically manifested by generic symptoms of meningeal irritation or convulsions, hemiparesis and

[A reversible posterior leukoencephalopathy syndrome in a patient with classical polyarteritis nodosa].

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We report a case of a reversible posterior leukoencephalopathy syndrome (RPLS). A 57-year-old male had classical polyarteritis nodosa with mononeuritis multiplex, renal insufficiency, and a high titer of p-ANCA (MPO). He was normotensive. He was treated with high dose methylprednisolone and then

Epilepsy: an anticipatory presentation of pediatric Wegener's granulomatosis.

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Wegener's granulomatosis is a necrotizing, granulomatous vasculitis that primarily affects the respiratory tract and kidneys. It is rare in children. Few pediatric and adult case reports described seizures and central nervous system involvement at initial presentation, and none described central

Hypertrophic pachymeningitis in a patient with Takayasu arteritis: One more association?

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Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory disease of the dura mater, described in association with various infections, systemic vasculitides such as Wegener's granulomatosis and giant cell arteritis. However, HP in association with Takayasu arteritis (TA) has not been

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