完全房室ブロックを合併した抗 signal recognition particle（SRP）抗体陽性ミオパチー

患者は70歳代前半女性．心不全で発症し，当院循環器内科で治療を受けた．心不全は利尿薬投与などで改善したが，CK 高値があり，その後に，首下がりや四肢近位筋筋力低下が出現進行したため，当科に入院した．右上腕二頭筋筋生検では，筋線維の大小不同，内部核の増加があり，壊死筋線維が多数みられたが，再生筋，myophagia や炎症細胞浸潤は軽度であった．抗体検査の結果と合わせ，抗signal recognition particle（SRP）抗体陽性ミオパチーと診断した．治療として，経口ステロイド療法を開始したが，治療開始17日目に失神発作を来たすようになった．心電図検査では完全房室ブロックであり，発作後３日目に永久ペースメーカー植え込み術を行った．経口ステロイド療法に加え，免疫グロブリン大量療法（IVIg 療法）を行ったところ，高CK 血症や頸部四肢筋力低下は徐々に改善した．抗SRP 抗体陽性ミオパチーの心合併症については，心伝導障害を含め，様々な議論があるが，これまでに完全房室ブロックを来たした報告はなく，貴重な症例と考えられた．A 70-year-old woman was admitted to our hospital owing to heart failure. She was treated and improved with diuretics. However, her serum CK values were persistently elevated throughout her hospitalization. She quickly developed head drop and showed muscle weakness, predominantly in the shoulders and pelvic girdles. A muscle biopsy from the right biceps brachialis revealed large variability in the myofiber size with many necrotic myofibers and few regenerative myofibers, as well as inflammatory cell infiltration. These findings combined with the immunological blood test led to a diagnosis of anti-SRP-positive myopathy, and she was treated with oral glucosteroids. However, she had frequent syncope attack 17 days after the beginning of treatment. Her ECG revealed complete atrioventral block, and she received a pacemaker implantation 3 days after the attack occurred. Her muscle weakness and hyperCKemia gradually improved by additional intravenous immunoglobulin treatment. The existence of cardiac complications of anti-SRP-positive inflammatory myopathy has been a matter of debate. Further extensive clinical observations will be required to clarify the clinical existence and the significance of anti-SRP antibodies on the pathogenesis of cardiac complications, including conduction block

Clinical value of abnormal median-normal sural sensory response pattern for the early diagnosis of acute oropharyngeal palsy: a comparison of recent and previous cases

　To evaluate whether nerve conduction study (NCS) findings are useful to make an early diagnosis acute oropharyngeal palsy (AOP). 　We evaluated 2 AOP patients. Patient 1: An 18-year-old male who developed a nasal voice two weeks after common-cold symptoms with no weakness in his extremities. Patient 2: A 16-year-old female who developed a nasal voice two days after diarrhea with no weakness in her extremities. We conducted a routine NCS on these 2 patients and followed up their changes. Both patients had an abnormal median-normal sural (AMNS) sensory response pattern, which improved over time. Both patients were diagnosed with AOP and treated with intravenous immunoglobulin (400 mg/kg/day) with a good prognosis. Anti-GQ1b and/or anti-GT1a IgG antibodies were identified in the serum of both patients a few weeks after their initial diagnoses. 　The AMNS sensory response pattern can be useful for the early diagnosis of AOP

Influence of placement sites of the active recording electrode on CMAP configuration in the trapezius muscle

Objective: We investigated how the active electrode placement site influences compound muscle action potential (CMAP) configuration of the upper trapezius muscle (TM). Methods: A nerve conduction study of the accessory nerve was performed, and the CMAPs obtained with two different placement sites, i.e., placement of the active recording electrode on the belly of the upper TM (CMAP-A) and placement of the electrode 2 cm behind the belly (CMAP-B), were compared. CMAPs were also obtained with the active recording electrode placed in the supraspinous fossa (CMAP-C). Results: All CMAPs were recorded from 21 healthy volunteers. The mean peak-to-peak amplitude of CMAP-B was 3.4 mV higher than that of CMAP-A (11.0 ± 4.0 mV vs. 14.4 ± 4.9 mV; P < 0.01). The mean peak-to-peak amplitude of CMAP-C was 10.3 ± 5.0 mV. Conclusions: CMAP of the upper TM was always higher when the active recording electrode was placed 2 cm behind the belly of the muscle. Significance: When stimulating the accessory nerve, a current spread occurs to the C5 spinal nerve root and another CMAP originating from the supraspinatus muscle occurs in the supraspinous fossa. The volume conduction from the supraspinatus muscle affects the active recording electrode on the TM, resulting in an increase in CMAP amplitude. Keywords: Active recording electrode, Compound muscle action potential, Nerve conduction study, Supraspinatus muscle, Trapezius muscle, Volume conductio

T Helper 17 Promotes Induction of Antigen-Specific Gut-Mucosal Cytotoxic T Lymphocytes following Adenovirus Vector Vaccination

Few current vaccines can establish antigen (Ag)-specific immune responses in both mucosal and systemic compartments. Therefore, development of vaccines providing defense against diverse infectious agents in both compartments is of high priority in global health. Intramuscular vaccination of an adenovirus vector (Adv) has been shown to induce Ag-specific cytotoxic T lymphocytes (CTLs) in both systemic and gut-mucosal compartments. We previously found that type I interferon (IFN) signaling is required for induction of gut-mucosal, but not systemic, CTLs following vaccination; however, the molecular mechanism involving type I IFN signaling remains unknown. Here, we found that T helper 17 (Th17)-polarizing cytokine expression was down-regulated in the inguinal lymph nodes (iLNs) of Ifnar2−/− mice, resulting in the reduction of Ag-specific Th17 cells in the iLNs and gut mucosa of the mice. We also found that prior transfer of Th17 cells reversed the decrease in the number of Ag-specific gut-mucosal CTLs in Ifnar2−/− mice following Adv vaccination. Additionally, prior transfer of Th17 cells into wild-type mice enhanced the induction of Ag-specific CTLs in the gut mucosa, but not in systemic compartments, suggesting a gut mucosa-specific mechanism where Th17 cells regulate the magnitude of vaccine-elicited Ag-specific CTL responses. These data suggest that Th17 cells translate systemic type I IFN signaling into a gut-mucosal CTL response following vaccination, which could promote the development of promising Adv vaccines capable of establishing both systemic and gut-mucosal protective immunity

Abnormal dip phenomenon: a characteristic electrophysiological marker in interdigital neuropathy of the foot Fenômeno da diminuição de amplitude anormal: um marcador eletrofisiológico característico da neuropatia interdigital do pé

OBJECTIVE: The nerve conduction findings in interdigital neuropathy of the foot (IDN; Morton's neuroma) have rarely been reported. We analyzed the nerve conduction data in 23 patients with suspected IDN studied between 1982 and 2002. METHOD: Diagnosis of IDN was made on the basis of clinical features. All patients underwent routine nerve conduction studies and a near-nerve needle sensory nerve conduction study of the interdigital nerves by Oh's method in the symptomatic foot. RESULTS:Of the 23 patients, the diagnosis of definite IDN was made in 13 cases and of possible NDN in the others cases. Nineteen were females. Twenty two patients had only one nerve affected. One patient had two nerves affected. The III-IV interdigital nerve was affected in 17 cases and the II-III interdigital nerve in 7 cases. The near-nerve needle nerve conduction showed abnormality in the affected interdigital nerves in all definite IDN cases and confirmed the diagnosis of IDN in 10 cases by the abnormal dip phenomenon (a selective decrease of 50% or more in the sensory CNAP amplitude of the affected nerve compared with that of the preceding interdigital nerve). In 11 possible IDN cases, IDN was identified by the abnormal dip phenomenon. CONCLUSION: The near-nerve needle sensory nerve conduction of the interdigital nerves is a highly sensitive diagnostic test, and abnormal dip phenomenon is the most characteristic electrophysiological marker for the diagnosis of IDN.<br>OBJETIVO: Os achados da condução nervosa na neuropatia interdigital do pé (NIP) têm sido raramente descritos. Nós analisamos os dados da condução nervosa de 23 pacientes com suspeita de NIP entre 1982 e 2002. MÉTODO: O diagnóstico de NIP foi clínico. Todos os pacientes foram submetidos a estudos de condução nervosa de rotina e ao estudo de condução sensitiva dos nervos interdigitais com agulha justa-neural pelo método de Oh. RESULTADOS: Dos 23 pacientes, o diagnóstico de NIP foi definitivo em 13 casos é possível nos demais. Dezenove pacientes eram mulheres e 22 tinham somente um nervo afetado. Apenas um paciente teve dois nervos comprometidos. O nervo interdigital III-IV foi afetado em 17 casos e o nervo interdigital II-III em 7 casos. A condução nervosa justa-neural foi anormal nos nervos interdigitais acometidos em todos os casos definitivos e confirmou o diagnóstico de neuropatia interdigital em 10 casos pelo fenômeno da diminuição de amplitude anormal (uma diminuição seletiva de 50% ou mais da amplitude do PANS do nervo afetado quando comparado com o nervo anterior. Em 11 casos possíveis, a neuropatia interdigital foi identificada pelo fenômeno da diminuição de amplitude anormal. CONCLUSÃO: A condução nervosa sensitiva justa-neural dos nervos interdigitais do pé é um teste diagnóstico altamente sensível e o fenômeno da diminuição da amplitude anormal é o marcador eletrofisiológico mais característico para o diagnóstico de neuroma de Morton