[A case of Lambert-Eaton myasthenic syndrome with exacerbation of respiratory failure triggered by acute myocardial infarction].

The patient, a 58-year-old man, experienced weakness of the proximal muscles in both lower extremities, and Lambert-Eaton myasthenic syndrome and small cell carcinoma of unknown primary origin were diagnosed. He received symptomatic treatment for myasthenia and radiochemotherapy for small cell carcinoma; once this regimen, the myasthenic symptoms improved. However, acute myocardial infarction occurred, after which type II respiratory failure developed, and the patient required ventilator management with tracheal intubation. Acute-phase treatment, such as plasma exchange, intravenous immune globulin therapy, and methylprednisolone pulse therapy, and intensification of symptomatic treatment allowed for extubation, and eventually the patient was able to walk independently. According to electrophysiological examination, compound muscle action potentials were larger at discharge than at the time of exacerbation.

Measurement of excitation-contraction coupling time in critical illness myopathy.

OBJECTIVE: This study aimed to develop a simple and reliable technique to assess excitation-contraction (E-C) coupling for early diagnosis of critical illness myopathy (CIM). METHODS: We prospectively performed clinical and electrophysiological examinations on patients admitted to intensive care unit (ICU). In addition to full neurological examinations and routine nerve conduction study, motor related potential (MRP) was recorded using an accelerometer attached to the base of hallux after tibial nerve stimulation, and E-C coupling time (ECCT) was measured from the latency difference between soleus compound muscle action potential (CMAP) and MRP. RESULTS: Of 41 patients evaluated, 25 met the criteria for ICU-acquired weakness, 23 of whom had CIM. The time to the first electrophysiological examination (time to first test) correlated negatively with CMAP and with MRP. Conversely, a positive correlation was observed between the time to first test and ECCT. E-C coupling impairment occurred in most of our patients with CIM by the third day of ICU admission, and prolonged ECCT could be the earliest detectable abnormality. CONCLUSIONS: The ECCT measurement is an easy and reliable technique to detect reduced muscle membrane excitability in the early stage of CIM. SIGNIFICANCE: The ECCT measured by our method using an accelerometer may be a parameter that predicts the development of CIM.

Streptococcus oralis Meningitis with Gingival Bleeding in a Patient: A Case Report and Review of the Literature.

An 81-year-old man with a history of gingival bleeding presented with a fever, headache, and drowsiness. His mouth and full dentures were unsanitary. Laboratory tests revealed Streptococcus oralis meningitis caused by odontogenic bacteremia. We reviewed eight reported cases, including the present case, because S. oralis meningitis is rare. Our review indicated that S. oralis meningitis needs to be considered when encountering cases of a fever, disturbance of consciousness, and headache with episodes of possible odontogenic bacteremia.

Severe Mononeuritis Multiplex due to Rheumatoid Vasculitis in Rheumatoid Arthritis in Sustained Clinical Remission for Decades.

Rheumatoid vasculitis (RV) usually occurs in patients with refractory rheumatoid arthritis (RA). An 80-year-old woman was transferred to our hospital because of muscle weakness and paresthesia in all 4 limbs. She had been diagnosed with RA 30 years ago and achieved sustained clinical remission. At presentation, polyarthritis and drop foot were observed, and rheumatoid factor was prominently elevated. A peripheral nerve conduction test revealed mononeuritis multiplex in her limbs. We suspected that RV had developed rapidly despite RA having been stable for many years and started immunosuppression therapy with steroids combined with azathioprine. The treatment prevented worsening of muscle weakness and paresthesia.

[Sporadic adult-onset neuronal intranuclear inclusion disease with abnormal electroretinogram, nerve conduction studies and somatosensory evoked potential].

A 70-year-old man, a urinary retention of unknown origin from 10 years ago, decreased cognitive function from 4 years ago, vision impairment advanced a year ago. Brain MRI with DWI showed high intensity erea in the cortico-medullary junction. We diagnosed as intranuclear inclusion body disease (NIID) because of p62-positive intranuclear inclusion bodies by skin biopsy. Electroretinogram revealed amplitude reduction in the cone response superiority. Nerve conduction test showed mild conduction velocity reduction. Furthermore, in the somatosensory evoked potential of the lower limb, latency of the first cortical component was prolonged. These electrophysiological abnormalities were considered to be associated with the pathological features of NIID.

Fisher syndrome with delayed facial weakness and taste impairment: a case report.

A 55-year-old man was admitted to our hospital because of acute onset of diplopia and gait disturbance. On admission, ophthalmoplegia, ataxia and areflexia were observed. He was diagnosed with Fisher syndrome and given intravenous immunoglobulin therapy from day 6 to day 10 after disease onset. After treatment, ophthalmoplegia and ataxia began to improve. However, he developed taste impairment on day 13 and right hemifacial weakness on day 16 after onset. A blink reflex test revealed right facial nerve impairment. On day 42 after onset, facial weakness and taste impairment remitted, and the blink reflex test result was normalized without additional treatment. Although it has been known that 10% of patients with Fisher syndrome complicated by delayed facial nerve palsy, the mechanism of the facial nerve palsy has not been elucidated. Therefore, this is a significant report to describe delayed facial nerve palsy combined with taste impairment and successive recordings of blink reflex and facial nerve conduction in a patient with Fisher syndrome.

Arachidonate 5-lipoxygenase establishes adaptive humoral immunity by controlling primary B cells and their cognate T-cell help.

In this study, we report the unique role of arachidonate 5-lipoxygenase (Alox5) in the regulation of specific humoral immune responses. We previously reported an L22 monoclonal antibody with which human primary resting B cells in the mantle zones of lymphoid follicles are well-defined. Proteomics analyses enabled identification of an L22 antigen as Alox5, which was highly expressed by naive and memory B cells surrounding germinal centers. Cellular growth of mantle cell lymphoma cells also seemed to depend on Alox5. Alox5(-/-) mice exhibited weak antibody responses specific to foreign antigens at the initial and recall phases. This was probably attributable to the low number of follicular and memory B cells and the functional loss of interleukin-21-mediated responses of follicular B cells. Moreover, Alox5(-/-) mice could not fully foster the development of follicular B helper T (Tfh) cells even after immunization with foreign antigens. Further experiments indicated that Alox5 affected mortality in experimentally induced enterocolitis in germ-prone circumstances, indicating that Alox5 would endow immunologic milieu. Our results illustrate the novel role of Alox5 in adaptive humoral immunity by managing primary B cells and Tfh cells in vivo.