Distal acquired demyelinating symmetric polyneuropathy progressing to classic chronic inflammatory demyelinating polyneuropathy and response to fludarabine and cyclophosphamide.

INTRODUCTION: Distal acquired demyelinating symmetric polyneuropathy (DADS) is proposed as a distinct entity from classic chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: We report a 58-year-old woman with DADS that progressed to a severe case of classic CIDP. RESULTS: She had distal numbness and paresthesias, minimal distal weakness and impaired vibratory sensation. She had anti-MAG antibodies, negative Western blot, and lacked a monoclonal gammopathy. There were prolonged distal motor latencies. She remained stable for 6 years until developing proximal and distal weakness. Nerve conduction studies showed multiple conduction blocks. She developed quadriparesis despite first-line treatment for CIDP. She was started on cyclophosphamide and fludarabine. Twenty-five months after receiving chemotherapy, she had only mild signs of neuropathy off all immunotherapy. CONCLUSIONS: DADS may progress to classic CIDP and is unlikely to be a separate disorder. Fludarabine and cyclophosphamide may be effective for refractory CIDP.

Case of Anti-Single Recognition Particle-Mediated Necrotizing Myopathy After Influenza Vaccination.

Immune-mediated necrotizing myopathy is a very rare inflammatory disease affecting skeletal muscles. Immune-mediated necrotizing myopathy may be associated with myositis-specific autoantibodies including anti-single recognition particle and anti-3-hydroxy-3- methylglutaryl-coenzyme A reductase, infectious agents (HIV or hepatitis C), other connective tissue disorders (such as scleroderma), and malignancy. We reported a 28-year-old healthy woman presented with subacute onset ascending muscle weakness 2 weeks after an annual influenza vaccination. Cerebral Spinal Fluid study showed normal cell counts with elevated protein and nerve conduction study showed reduced diffuse compound muscle action potential amplitudes suggesting a diagnosis of Guillain-Barré syndrome. Despite treatment using intravenous immunoglobulin, her condition continued to get worse with new bulbar and respiratory muscle weakness. Eventually, the diagnosis of anti-single recognition particle-mediated necrotizing myopathy was made based on elevated creatine kinase, thigh magnetic resonance imaging, muscle biopsy, and positive antibody testing. Our patient responded to the combination of intravenous immunoglobulin, prednisone, and anti-CD20 monoclonal antibody, rituximab.

Corrigendum to "A Case of Sporadic Creutzfeldt-Jakob Disease Presenting as Conversion Disorder".

[This corrects the article DOI: 10.1155/2017/2735329.].

Effect of emplaced nZVI mass and groundwater velocity on PCE dechlorination and hydrogen evolution in water-saturated sand.

The effect of nZVI mass loading and groundwater velocity on the tetrachloroethylene (PCE) dechlorination rate and the hydrogen evolution rate for poly(maleic acid-co-olefin) (MW=12K) coated nZVI was examined. In batch reactors, the PCE reaction rate constant (3.7×10-4Lhr-1m-2) and hydrogen evolution rate constant (1.4 nanomolLhr-1m-2) were independent of nZVI concentration above 10g/L, but the PCE dechlorination rate decreased and the hydrogen evolution rate increased for nZVI concentration below 10g/L. The nonlinearity between nZVI mass loading and PCE dechlorination and H2 evolution was explained by differences in pH and Eh at each nZVI mass loading; PCE reactivity increased when solution Eh decreased, and the H2 evolution rate increased with decreasing pH. Thus, nZVI mass loading of <5g/L yields lower reactivity with PCE and lower efficiency of Fe° utilization than for higher nZVI mass loading. The PCE dechlorination rate increased with increasing pore-water velocity, suggesting that mass transfer limits the reaction at low porewater velocity. Overall, this work suggests that design of nZVI-based reactive barriers for groundwater treatment should consider the non-linear effects of both mass loading and flow velocity on performance and expected reactive lifetime.

A Case of Sporadic Creutzfeldt-Jakob Disease Presenting as Conversion Disorder.

Background. Creutzfeldt-Jakob disease is a rare disorder of the central nervous system. Its initial diagnosis may be obscured by its variable presentation. This case report illustrates the complexity of diagnosing this disease early in the clinical course, especially when the initial symptoms may be psychiatric. It offers a brief review of the literature and reinforces a role for consultation psychiatry services. Methods. PUBMED/MEDLINE was searched using the terms "Creutzfeldt-Jakob disease", "psychiatric symptoms", "conversion disorder", "somatic symptom disorder", "functional movement disorder", and "functional neurologic disorder". Case. The patient was a 64-year-old woman with no prior psychiatric history who was initially diagnosed with conversion disorder and unspecified anxiety disorder but soon thereafter was discovered to have Creutzfeldt-Jakob disease. Discussion. This case highlights the central role of psychiatric symptoms in early presentations of Creutzfeldt-Jakob disease. Still, few other cases in the literature report functional neurological symptoms as an initial sign. The consultation psychiatrist must remain alert to changing clinical symptoms, especially with uncharacteristic disease presentations.

Difficult diagnosis: strangulated obturator hernia in an 88-year-old woman.

The obturator hernia is a rare type of hernia that can present a diagnostic challenge for the clinician. We report a case of an 88-year-old woman who presented with a history of right iliac fossa pain, bilious vomiting and diarrhoea. Non-specific findings on examination and blood tests made the diagnosis difficult, however, a CT scan of her abdomen revealed the site of the obstruction and the patient was taken to theatre for emergency surgery. We review the literature with focus on the diagnosis of obturator hernias and the different surgical approaches used. The authors believe that this case is of educational value to healthcare professionals, particularly those working in general practice, emergency departments and on surgical teams. It highlights to doctors that patients with incarcerated obturator hernias can present with or without overt signs of intestinal obstruction and emphasises the fact that an obturator hernia can be an important cause of intestinal obstruction in a thin, elderly woman.

Fludarabine in the Treatment of Refractory Chronic Inflammatory Demyelinating Neuropathies.

Inflammatory demyelinating neuropathies have variable responses to immunomodulating therapy. Eight patients with chronic inflammatory neuropathies who were refractory to standard therapy were treated with fludarabine, a combination of fludarabine and cyclophosphamide, and in 1 case with fludarabine and rituximab. Five patients with immunoglobulin M anti-myelin-associated glycoprotein neuropathies received fludarabine. Three patients with chronic inflammatory demyelinating polyneuropathy received a combination of fludarabine and cyclophosphamide. All 8 patients improved in either functional status or strength with minimal toxicities. Most patients experienced sustained remission after the use of fludarabine or fludarabine and cyclophosphamide. Fludarabine alone or in combination with cyclophosphamide should be considered for patients with inflammatory demyelinating neuropathies, refractory to other treatments.