Distal acquaried demylinating symmetric neuropathy in two patients with essential hiperIgEmia

Abstract

Several rare cases of polyneuropathy have been described recently. There is a very large and differential diagnosis for polyneuropathy. Epidemiology of this condition is still not exact 1 . We herein report two cases of a 67-year-old man and a 42-year-old woman with subacute progressive clinical picture of polyneuropathy and hyperIgEaemia. Those patients presented with similar symptoms, such as distal tetraparesis grade 4, generalized hyporeflexia, poor imbalance, decreased sensation of pain and temperature in the extremities, fine distal tremor and intense pain of the fingers of both hands and feet. Both showed more than 1000 ku/L of IgE in serum exams and have asthma. No other monoclonal gammopathy were found. The more common causes of polyneuropathy, like diabetes, hypothyroidism, B12 hypovitaminosis, infections like human T lymphotropic virus (HTLV 1 and 2), hepatitis C virus (HCV) and human immunodeficiency virus (HIV 1 and 2), were excluded, and there was no nerves enlargement in their physical exam. Nerve conduction studies revealed a conduction block amplitudes in motor (CMAP), lower terminal latency index (TLI) and alterations in sensory (SNAP) responses. In the first exam, we don't found SNAP responses, and the other electroneuromyography done showed small amplitude SNAP responses; prolongaded latency in nerves of upper limbs and both sural nerves were abolished (Table). Bilateral fibrillations or positives sharp waves were found in tibial anterior, gastronomies, paravertebral lumbar and cervical, gluteus medium, biceps and triceps. In both patients, the histologic features on sural nerve biopsy described no evidence of subperineurial or endoneurial edema or mononuclear inflammatory cell infiltrate in the epineurium or endoneurium. Those nerve biopsy describes mild loss of myelinated fibers and minimal axonal degeneration, and showed a slightly decrease number of thinly myelinated large caliber axons, with small onion bulb formations.We suggest that IgE-mediated allergy may be one potential cause of distal acquired demylinating symmetric neuropathy (DADS) ) weekly in the first month and a single dose by month control power and pain of male patient. Only one cycle of intravenous immunoglobulin (0.5 mg/kg/day) for five days do not demonstrated to be effective for female. The best pain treatment for female patient is using 60 mg of oxycodon four times a day. Dysimmune neuropathy is an etiologically heterogeneous entity with diverse clinical presentations. Conventional treatment options, including corticosteroids, intravenous immunoglobulin, or plasma exchange, often fail to treat dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy and monoclonal gammopathy with its subtypes. Therefore, a significant percentage of patients require adjunctive immunosuppressive therapies. Currently, several monoclonal antibodies have been tested in open-label small-sized studies or even in single cases so as to establish future directions in the therapy of diseases o...

Guillain-Barré syndrome associated with hyper-IgE-emia

“…Although peripheral nerve involvement is not a well-known complication, a few cases of hyper-IgE-emia associated with subacute or chronic polyneuropathy have been reported. 3,4 However, to our knowledge this is the first case of GBS associated with hyper-IgE-emia. It is possible that GBS and the hyper-IgE-emia in this case were coincidental.…”