Neurologic Manifestations of Schoenlein-Henoch Purpura: Report of Three Cases and Review of the Literature

Abstract

Three patients developed prominent neurologic symptoms and signs associated with Schoenlein-Henoch purpura. A 71/2-year-old boy was seen with status epilepticus after a 2-week history of generalized headaches, irritability, and intermittent colicky abdominal pain. A left hemiparesis and a left homonymous hemianopia with a right gaze preference that were present on initial examinations gradually resolved, but a mild left arm paresis persisted. Cutaneous, renal, and joint involvement followed initial CNS manifestations. The second patient, a 7-year-old girl, had a complex partial seizure with secondary generalization and a postictal hemiparesis seven days after presentation with classic signs of Schoenlein-Henoch purpura. Behavioral changes were noted during the acute phase of the illness. The third patient, a 13-year-old boy, developed signs of a left brachial plexopathy and transient weakness of his right leg during a complicated course of Schoenlein-Henoch purpura. Review of the world literature indicates that headaches and mental status changes are the most frequent neurologic complications of Schoenlein-Henoch purpura, followed by seizures, focal neurologic deficits, mononeuropathies, and polyradiculoneuropathies. The vasculitis of Schoenlein-Henoch purpura can involve the nervous system and may add significantly to the morbidity of the illness.

Steroid-resistant peripheral neuropathy in a child: a rare finding in immunoglobulin a vasculitis

“…3 Approximately 3 weeks later, while he was hospitalized on steroid treatment for gastrointestinal and scrotal involvement, peripheral neuropathy developed which is a finding that is also very rarely reported in IgAV. 5 Other systemic vasculitis should be considered in differential diagnosis of a patient with purpura and neuropathy. Skin biopsy findings are also important to exclude other forms of vasculitis such as ANCA-associated vasculitis.…”

“…Belman et al 5 reported that nervous system involvement in IgAV is usually underestimated. Garzoni et al 2 reviewed nervous system dysfunction in IgAV and showed that peroneal neuropathy, peripheral facial palsy, Guillain-Barre´ syndrome, brachial plexopathy, posterior tibial nerve neuropathy, femoral neuropathy, ulnar neuropathy and mononeuritis multiplex were reported as cranial or peripheral neuropathy conditions.…”

Fokale Krampfanfälle und Posteriores Reversibles Enzephalopathie-Syndrom (PRES) als Erstmanifestation einer IgA-Vaskulitis

“…Obwohl die Langzeitprognose der IgAV hauptsächlich auf die Nierenschädigung zurückzuführen ist, kann eine zerebrale Beteiligung zu einer erheblichen Morbidität und Mortalität führen 13 . Schwere neurologische Symptome wurden in 2–8 % der IgAV-Fälle berichtet 19 , 35 , 37 . Neuere Studien wie z.…”

“…Obwohl Krampfanfälle vorwiegend in der akuten Phase der Erkrankung auftreten, haben wir in der Literatur 3 Patienten gefunden, bei denen Krampfanfälle das 1. Anzeichen einer IgAV waren 8 , 17 , 19 . Wir kommen daher zu dem Schluss, dass IgAV als Differenzialdiagnose von afebrilen Krampfanfällen in Betracht gezogen werden sollte, insbesondere bei normothrombozytären Petechien trotz fehlender ZNS-Entzündung.…”

“…Zusammenfassend kommen wir zu dem Schluss, dass schwere ZNS-Beteiligungen wie Krampfanfälle und PRES unterschätzte Merkmale der IgAV sind, auf die Ärzte achten sollten. Darüber hinaus weisen unser Fall und 3 frühere Berichte 8 , 17 , 19 darauf hin, dass ZNS-Symptome anderen Manifestationen der IgAV vorausgehen können. So kann die IgAV zunächst andere Erkrankungen wie das Waterhouse-Friderichsen-Syndrom imitieren.…”