Unusual Domestic Source of Lead Poisoning

Non-occupational lead poisoning is not rare, mainly occurring in domestic situations in children, but also in adults. Lead poisoning was observed in a 65 years-old woman non-exposed to risk that caught our attention with a diagnostic suspicion of acute intermittent porphyria according to recurrent episodes of abdominal pain and neuropathy of upper limbs. Acute intermittent porphyria was excluded by a laboratory investigation that showed instead severe lead poisoning. After several thorough examinations of the domestic environment, the source of intoxication has been detected in some cooking pots that released high concentrations of lead. Ethylenediamine tetracetic acid disodium calcium therapy (three cycles) reduced consistently blood lead concentration and, after one year, neuropathy was almost entirely recovered

Leukocytoclastic vasculitis associated with multifocal sensory neuropathy responsive to intravenous immunoglobulins: a case report

Leukocytoclastic vasculitis (LCV) is a common form of small-vessel vasculitis, which commonly presents as palpable purpura or petechiae, caused by deposition of circulating immune complexes on vessels walls that attracts granulocytes which damage the vascular endothelium and leading to erythrocytes extravasation. The skin is the most commonly involved organ, but also renal, gastrointestinal, pulmonary, cardiovascular and neurological systems may be affected. Skin lesions may be the initial signs of systemic vasculitis. Systemic symptoms may be present, such us fever, myalgia, abdominal pain and arthralgia. The presence of neuropathy/mononeuritis multiplex is expression of severe vasculitic involvement. Herein, we describe the case of a patient with leucocytoclastic vasculitis associated to sensitive neuropathy, responsive to intravenous immunoglobulins (IVIg) therapy, after the failure of classic systemic treatments

Obinutuzumab, a new anti-CD20 antibody, and chlorambucil are active and effective in anti-MAG antibody polyneuropathy

Rituximab, a chimeric anti-CD20 monoclonal antibody (mAb), has been used in polyneuropathy associated with anti-MAG antibody polyneuropathy with controversial results. Herein, we report on two patients with anti-MAG antibody neuropathy and concurrent chronic lymphocytic leukemia (CLL), who dramatically responded to obinutuzumab, a novel glycoengineered humanized anti-CD20 mAb

The BCL2 Inhibitor Venetoclax Plus Rituximab Is Active in MYD88 Wild-Type Polyneuropathy With Anti-MAG Antibodies

Objectives: Ibrutinib is active in anti-myelin-associated glycoprotein (MAG) polyneuropathy with MYD88L265P mutation; however, its efficacy is likely to be low in MYD88 wild-type patients. Venetoclax, an oral inhibitor of BCL2, in combination with rituximab is highly active in ibrutinib-resistant hematologic malignancies. We report on the first patient with anti-MAG polyneuropathy and MYD88 wild-type who responded to venetoclax-rituximab. Methods: A 62-year-old woman with chronic lymphocytic leukemia had IgM/K anti-MAG neuropathy. She needed bilateral support to walk outdoors, despite therapy with rituximab/cyclophosphamide. Tremor and symptoms at arms prevented her capability of performing common tasks. Bone marrow genetic showed lack of MYD88 mutation. Venetoclax was given orally starting from 20 mg up to 400 mg for 24 months. Rituximab was administrated IV, after the ramp-up phase, at 375 mg/m2 for the second month and then monthly at 500 mg/m2 for months 3-7. Results: After 12 months of venetoclax IgM levels decreased from 1.16 to 0.52 g/L, the paraproteins became undetectable and anti-MAG antibody titer decreased. The patient regained the capability of walking independently. Tremor disappeared, she is back able to write and knitt. Discussion: The first patient with anti-MAG neuropathy treated with venetoclax-rituximab shows encouraging results. Classification of evidence: This study provides Class IV evidence that for a patient with relapsed anti-MAG antibody polyneuropathy, MYD88 wild-type, venetoclax plus rituximab is effective

'Snake eyes' MRI sign: possible role of cobalt toxicity?

‘Snake eyes’ sign is the magnetic resonance imaging (MRI) occurrence of bilateral hyperintensities of the anterior horns of spinal cord on axial T2-weighted MR images. This neuroradiological sign has been commonly attributed to lower motor neuron (LMN) syndromes, such as Hirayama disease, but also in cervical spondylotic myelopathy and spinal cord infarction. We report on a patient with upper limb LMN syndrome and MRI snake eyes sign secondary to cobalt neurotoxicity