Electromyography varies by stage in inclusion body myositis.

Introduction: Inclusion body myositis (IBM) is a chronic inflammatory muscle disease that is characterized by mixed myogenic and neurogenic electromyography (EMG) findings. We investigated the association between EMG findings and the IBM stage. Methods: We included consecutive patients diagnosed with IBM based on muscle biopsy and had needle EMG performed within 1 month of biopsy. Motor unit potential waveform (MUP) in EMG and pathological findings were compared between patients in early and late phases. Results: In total, 30 patients with biopsy-confirmed IBM and 254 muscles were included. The rate of abnormal discharge did not differ according to disease stage. There was a difference in the frequency of occurrence between myogenic suggestive MUP and neurogenic of biceps and flexor digitorum profundus in the late phase. Abnormal MUP was observed even in muscles without muscle weakness, and myogenic changes were predominant in biceps and gastrocnemius with muscle weakness. The biopsy findings on the contralateral side of the muscle where electromyography was performed revealed a tendency for muscles that exhibited myogenic origin to have more inflammatory cells and RV; however, the difference was not significant. Conclusion: The target muscles for EMG must be selected considering the disease stage as well. In the early stages of IBM, EMG results should be interpreted cautiously, as neurogenic suggestive pattern of MUP might also be exhibited. Contralateral electromyography findings may be helpful in selecting muscles for muscle biopsies, such as biceps and quadriceps.

Walking Disability in Patients with Pelvic Insufficiency Fracture after Radiotherapy for Uterine Cervical Cancer.

OBJECTIVE: Radiotherapy is an essential component of curative or palliative therapy for patients with uterine cervical cancer. Although advances in radiotherapy have led to longer survival, survivors may consequently be at risk of pelvic insufficiency fracture (PIF). We retrospectively reviewed medical records and clinical outcomes to assess the impact of PIF on walking disability. METHODS: Between January 2002 and December 2009, 145 uterine cancer patients treated with radiotherapy in our hospital were reviewed. Among these, 15 patients (10.3%) were diagnosed with PIF. The types of fractures were identified according to the AO/OTA classification system. Medical records were examined to establish the time to first diagnosis of PIF, the type of fracture, and clinical outcomes. Disability was assessed using Barthel index mobility scores. RESULTS: The median time to PIF detection was 16 months. Of the 15 patients with PIF, 14 had type B fractures (7 cases of B2 and 7 cases of B3) and 1 had a type C fracture. Among 11 patients with pelvic pain, 6 achieved pain control but 5 patients with bilateral lesions in the posterior arch or lateral compression of the sacrum developed pain that finally resulted in walking disability and a lower performance status. CONCLUSIONS: PIF causes severe motor disturbance in patients with unstable fracture types. Routine imaging checkups were useful during the 5 years after completion of radiotherapy; in nine patients the fracture progressed for longer than 1 year. In cancer rehabilitation for PIF patients, continuous assessment is essential for predicting walking disability.

Preoperative low-dose steroid can prevent respiratory insufficiency after thymectomy in generalized myasthenia gravis.

BACKGROUND: Postoperative respiratory insufficiency (PRI) in myasthenia gravis (MG) often occurs within several days after thymectomy and remains problematic. In limited studies reporting that preoperative steroids prevented PRI in patients with MG, high doses of steroids were used and detailed information on the use of steroids is limited. Because high-dose steroids significantly increase the risk of adverse effects, we studied 37 patients with generalized MG to investigate whether low-dose steroids might prevent PRI. METHODS: The low-dose steroids were started orally, and the dose was gradually increased to the maximum level (30 mg/day). Immediately before thymectomy, patients received the maximum dose of oral steroids daily. PRI was defined as the development of restrictive dysfunction requiring mechanical ventilation within 3 days after thymectomy and total postoperative mechanical ventilation support time of >24 h. RESULTS: The rate of PRI in the low-dose steroid use group was significantly lower than that in the no-steroid use group. The postoperative stay in the intensive care unit was shorter in the steroid use group. CONCLUSIONS: Extended thymectomy is a well-accepted surgical treatment for selected patients with MG. However, PRI remains problematic. Our results suggest that not only preoperative high-dose steroid treatment, but also low-dose steroid treatment can prevent PRI.

Clinical outcomes and serum uric acid levels in elderly patients with amyotrophic lateral sclerosis aged ≥ 70 years.

BACKGROUND: Amyotrophic lateral sclerosis is a slowly progressive fetal neurodegenerative disease in which clinical phenotype and nutritional status are considered prognostic factors. Advanced age has also been reported to carry a poor prognosis in amyotrophic lateral sclerosis. The elderly population is expected to increase in Japan, as well as in other countries in the near future. Whether late-onset amyotrophic lateral sclerosis affects the average lifespan or survival of patients and the nutritional status was related to survival remains an open question. METHODS: We studied the survival of elderly 34 patients with clinically definite amyotrophic lateral sclerosis aged ≥ 70 years and investigated serum triglycerides, cholesterol, LDL/HDL ratio, and glucose. Serum uric acid was examined. RESULTS: The average age at respiratory disorders or death as a whole was 77.5 ± 4.3 years. Survival did not differ significantly between different clinical phenotypes or between patients with and those without riluzole usage. Survival differed significantly between patients with and those without other complications. No biochemical parameter is correlated with outcome in this series, including elevated triglyceride or cholesterol levels and an increased LDL/HDL ratio. The survival correlated with the serum uric acid level (r = 0.407, p = 0.017). CONCLUSIONS: The onset of amyotrophic lateral sclerosis at ≥ 70 years of age might not be the key determinant of survival in patients with amyotrophic lateral sclerosis.

Paradoxical gait at a narrowed entrance in a patient with Hoehn-Yahr stage III Parkinson's disease.

Recently, we studied fallers and non-fallers with Hoehn-Yahr stage III Parkinson's disease (PD) using a path that suddenly narrowed, which we originally designed and produced. A risk of future falls was suggested to be related to slow gait with freezing (SGF) elicited by a fear of falling before arrival at a narrowed entrance or while walking on a narrow path, as well as to the Unified Parkinson's Disease Rating Scale part II score, associated with SGF. In the same study, we had faller patients walk on a path that narrowed in a straight-line fashion to determine whether SGF could be improved. In one patient, who showed a unique paradoxical gait, SGF resolved. We describe this patient in the hope that our experience will provide potential clues to effective ways to prevent future falls in patients with Hoehn-Yahr stage III PD. To prevent gait instability elicited by fear of falling in patients with Hoehn-Yahr stage III PD, it might be useful to remove narrowed entrances.

Clinical analysis and outcomes of amyotrophic lateral sclerosis with demyelinating polyneuropathy.

Abnormalities of both motor and sensory nerve action potentials, similar to those found in demyelinating polyneuropathy, may occur in patients with amyotrophic lateral sclerosis (ALS). We analyzed the clinical features of unusual ALS patients with demyelinating polyneuropathy (DPN) to delineate the characteristics and outcomes of this rare condition. We reviewed three ALS patients with DPN who were confirmed to meet the electrophysiological nerve conduction criteria for DPN among 157 patients with ALS. At the initial neurological examination, one patient had both subjective sensory symptoms and abnormal results of sensory examinations, and one patient had sensory symptoms. Motor weakness of the limbs was present in all patients, and fasciculation was present in two patients. Anti-GalNAc-GD1a IgG antibodies were evident in one. Sural nerve biopsy showed a moderate, marginal reduction in myelin thickness, and teased fiber analysis revealed segmental demyelination and remyelination, but axonal degeneration was found in one patient. The mean interval from disease onset to respiratory failure or death in our three patients and seven previously documented ALS patients with DPN was 43.1 ± 18.7 months. Our findings suggest that survival in ALS with DPN is similar to that in classic ALS.

Isolated inflammatory myopathy with rimmed vacuoles presenting with dropped head.

We describe an unusual case of inflammatory myopathy with rimmed vacuoles associated with dropped head syndrome. Muscle biopsy in our patient revealed variations in fiber size with fiber necrosis and regeneration, accompanied by many rimmed vacuoles and areas of endomysial cell infiltration. Electron microscopy demonstrated autophagic vacuoles and tubulofilamentous inclusions. This myopathy can cause dropped head syndrome in a subgroup of patients.

Cerebral Sinus and Venous Thrombosis Associated with von Willebrand Factor, Independently of Factor VIII.

BACKGROUND AND PURPOSE: Previous studies have linked procoagulant factor VIII (F VIII) to an increased risk of venous thrombosis, whereas the relation between plasma von Willebrand factor (VWF) and venous thrombosis remains poorly understood. Elevated VWF levels are frequently found in patients with cerebral sinus and venous thrombosis (CSVT), always in association with high F VIII levels. We describe a patient with CSVT accompanied by elevated VWF levels without high F VIII levels. CASE DESCRIPTION: A 23-year-old healthy man who had headache noticed difficulty in moving the right hand. On the following day, he lost consciousness and had partial seizures of the right hand. After regaining consciousness, weakness of the right extremities developed. The cranial angiogram confirmed occlusion of the superior sagittal sinus. The levels of VWF and F VIII were 238% and 101.9 IU/dl, respectively. We performed balloon percutaneous transluminal angioplasty and mechanical thrombectomy, leading to successful recanalization of the intracranial sinuses. VWF levels were decreased along with radiographic improvement, independently of F VIII. CONCLUSION: VWF may contribute to CSVT and that inhibition of VWF activity potentially has a role in the future treatment of pathological conditions related to venous thrombosis.