No abstract

Background: SPS is a disorder consisting of rigidity of axial muscles with painful spasms. More than 80 % of SPS patients have high titer antibodies against glutamic acid decarboxylase (GAD). The use of rituximab for the treatment of SPS is a recent therapeutical approach showing promising results. We present a case of SPS treated with rituximab, showing a good and safe response. Case: A 38-year-old female patient presented with a history of rigidity of abdominal and paravertebral muscles associated with painful spasms in lower back region, increased tonus, lumbar lordosis, frequent falls and severe functional limitation. The anti-GAD antibodies were positive in high titles. Electromyography showed continuous motor activity with normal morphology especially on paravertebral muscles. She had a partial response to baclofen and diazepam, but could not tolerate it because of somnolence, and started the treatment with rituximab. After one year, the baclofen was discontinued and the diazepam reduced. The axial stiffness and spasm frequency improved, including postural instability, without new episodes of falls. Discussion: Rituximab is a monoclonal antibody targeting the CD20 antigens on the surface of mature B lymphocytes. After binding to these antigens, it initiates a cascade of biochemical events leading to apoptosis. Its use has been approved for numerous diseases with promising results. The use of rituximab in the treatment of SPS is a recent approach and good results have been reported. Conclusion: Rituximab may be a promising option in SPS treatment. However, this is a preliminary paper showing partial results requiring long-term follow-up.

Background: The long incubation period of leprosy, its insidious signs and symptoms produce difficulties in its diagnosis and correct clinical classification. The early recognition of neural impairment in leprosy, especially in household contacts with subclinical infection and in the primary neural form, in which the classic clinical and laboratory findings of the disease are, by definition, absent, represents a major challenge in clinical practice. Objectives: Characterize the clinical, molecular, serological and neurophysiological aspects in the early diagnosis of leprosy neuropathy, in household contacts with subclinical infection (positive ELISA anti-PGL1 serology. Design and setting: Longitudinal study carried out at the Clinics Hospital - Federal Univeristy of Uberlândia, a center specialized in Leprosy/Sanitary Dermatology. Methods: 361 seropositive household contacts (CDSP), defined as subclinical infection, were recruited, followed up at a national referral center for leprosy in Brazil, from 2014 to 2016. All individuals underwent a clinical, laboratory and neurophysiological evaluation. Results: 361 CDSP were evaluated. The qPCR analysis was positive in 35.5% (128/361) in the dermal shaving and in 25.8% (85/361) in the skin biopsy of the CDSP. In the electroneuromyographic evaluation, 23.5% (93/361) of the CDSP showed signs of neural involvement, with an average of 2.1 nerves compromised by CDSP. 62.3% (53/93) presented a pattern of mononeuropathy in ENMG. Conclusions: Annual monitoring of CDSP, a prevalence of peripheral neural impairment assessed by ENMG, favoring early treatment.

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Background: Peripheral neuropathies in cancer are most often due to neurotoxic chemotherapeutic agents. Approximately 30% of patients receiving neurotoxic chemotherapy (CTX) will suffer from chemotherapy-induced peripheral neuropathy (CIPN). Paclitaxel is an extremely effective chemotherapeutic agent for the treatment of breast, ovarian, and lung cancer. However, paclitaxel-induced peripheral neuropathy occurs in 59-87% of patients who receive this drug. Paclitaxel is an anti-tubulin drug that causes microtubule stabilization, resulting in distal axonal degeneration, secondary demyelination and nerve fiber loss. Case: We present a case of a 68-year-old female patient with history of breast cancer who presented sensorial ataxia and progressive muscle weakness two months after starting CTX with paclitaxel. The physical examination showed tetraparesis with proximal predominance, areflexia, severe hypopalesthesia and postural instability. Electroneuromyography showed the existence of asymmetric demyelinating polyradiculoneuropathy, with conduction block and temporal dispersion in practically all evaluated nerves. The cerebrospinal fluid confirmed the albumin-cytological dissociation. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was confirmed and patient underwent monthly treatment with methylprednisolone with good response. Discussion: Evidences has implicated neuroinflammation in the development of PIPN. While most CTX drugs do not cross the blood-brain-barrier, they readily penetrate the blood-nerve-barrier and bind to and accumulate in dorsal root ganglia and peripheral axons. CTX can induce neuroinflammation through activation of immune and immune- like glial cells. In fact, immune cells (e.g., macrophages, lymphocytes) and glial cells (e.g., Schwann cells) in the peripheral nervous system play important role in the induction and maintenance of neuropathy. Conclusion: CIDP should be included in the spectrum of CIPN.