Zahra Baghestani; Reza Boostani
Abstract
A 43-year-old male presented with diplopia and right sixth-nerve palsy. Brain magnetic resonance imaging (MRI) demonstrated a lesion in the right periventricular area. High-dose corticosteroid pulse therapy did not resolve the symptom. After one month, his diplopia progressed and he developed weakness ...
Read More
A 43-year-old male presented with diplopia and right sixth-nerve palsy. Brain magnetic resonance imaging (MRI) demonstrated a lesion in the right periventricular area. High-dose corticosteroid pulse therapy did not resolve the symptom. After one month, his diplopia progressed and he developed weakness of the left lower limb. Detailed examination revealed left sixth-nerve palsy, dropped foot, waddling gait, atrophy of the gluteal muscles and mild atrophy and weakness of the right upper limb. Neurological examination supported evidence of multiple cranial nerve palsies along with asymmetrical peripheral neuropathy. Electrodiagnostic studies were compatible with a mononeuritis multiplex. Rheumatologic evaluations were normal. Malignancy work-up were normal, except for some insignificant lymph nodes. Bone marrow aspiration and biopsy were normal. The second brain MRI detected multiple homogenous enhancing lesions in the right periventricular area.The result of stereotactic biopsy and immunohistochemistry staining demonstrated primary B-cell CNS lymphoma (PCNSL). Mononeuritis multiplex has not been reported as a paraneoplastic manifestation of PCNSL yet. In other words, it is not clear whether involvement of the peripheral nervous system in our patient is a paraneoplastic manifestation of PCNSL or a coincidence of PCNSL and hematologic lymphoma presenting with peripheral vasculitic neuropathy. It is recommendedthat future studies focus more on symptoms associated with PCNSL to recognize the exact relationship between PCNSL and peripheral neuropathy.
Abdolreza Malek; Forough Rakhshanizadeh; Sheila Kianifar
Abstract
Churg-Strauss syndrome (CSS) is a type of vasculitis of small-to-medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotizing vasculitis and peripheral blood hypereosinophilia. It is currently called eosinophilic granulomatosis with polyangiitis (EGPA). This ...
Read More
Churg-Strauss syndrome (CSS) is a type of vasculitis of small-to-medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotizing vasculitis and peripheral blood hypereosinophilia. It is currently called eosinophilic granulomatosis with polyangiitis (EGPA). This disease affects both genders and all age groups, but it is very rare among children. CSS diagnosis is based on clinical findings such as asthma, eosinophilia, rhinosinusitis, and signs of vasculitis in major organs. In cases where steroids alone or in combination with other immunosuppressive agents are used as treatment, the outcome and long-term survival are usually satisfying. In comparison with other types of systemic vasculitis, the mortality rate of this syndrome is low. In this study, we present the case of a 7-year-old boy with poorly controlled bronchial asthma since three years of age. This case had developed purpuric skin lesions, sinusitis, arthritis, and weakness of the limbs with symptoms of mononeuritis multiplex at the age of seven. After being admitted to our hospital, a series of studies, including complete blood count-diff, chest X-ray, paranasal sinus radiography, brain magnetic resonance imaging, nerve conduction study, spirometry, and serological tests for autoantibodies, were performed and he was diagnosed with CSS. Thereafter, he received regular corticosteroid therapy in combination with methotrexate, and his symptoms were generally well-controlled with the beginning of the treatment. The clinical characteristics, diagnosis, and management of CSS in children are also reviewed in this study.