Showing papers by "Yu-ichi Noto published in 2009"

Utility of the distal compound muscle action potential duration for diagnosis of demyelinating neuropathies.

Abstract: To assess the significance of distal compound muscle action potential (CMAP) duration for diagnosis of demyelinating neuropathies, electrophysiologic data were reviewed from 471 subjects, including 145 normal controls, 60 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 205 with other neuropathy, and 61 with amyotrophic lateral sclerosis (ALS). The duration of distally evoked CMAP was measured in the median, ulnar, tibial, and peroneal nerves. Optimal cut-off values were calculated with receiver-operating characteristic (ROC) curves. In comparison of normal controls and CIDP patients, ROC analyses showed the sufficient area under the curves (82-93%). When the cut-off values in the detection of demyelination were determined as the point with 98% specificity vs. normal on the ROC curves (median, 6.6 ms; ulnar, 6.7 ms; peroneal, 7.6 ms; tibial, 8.8 ms), the sensitivity was 77% for CIDP, with a specificity of 90% vs. ALS and 95% vs. diabetic neuropathy. The distal CMAP duration is a useful index for the detection of distal demyelination. We suggest the above cut-off values for each nerve as one of the electrodiagnostic criteria for demyelinating neuropathies, preferentially affecting the distal nerve terminals, such as CIDP.

Cardiomyopathy in a Japanese family with the Glu61Lys transthyretin variant: a new phenotype

Abstract: We report a Japanese family with transthyretin (TTR) amyloidosis caused by ATTRGlu61Lys that was associated with progressive cardiomyopathy, peripheral neuropathy, and bilateral carpal tunnel syndrome. Amyloidotic polyneuropathy in association with ATTRGlu61Lys was previously described in a Japanese family by Shiomi et al., (Biochem Biophys Res Commun 1993;194:1090–1096), and the main clinical features of that family were late-onset sensorimotor polyneuropathy and severe autonomic disturbance. However, there have been no descriptions of either cardiac involvement or carpal tunnel syndrome. The Japanese family, we present here, had severe cardiomyopathy as a cardinal symptom, which is a new phenotype of the TTR amyloidosis with ATTRGlu61Lys. This report, alongwith a previous one, demonstrates the clinical variety of TTR amyloidosis caused by ATTRGlu61Lys.

[Subacute sensory neuronopathy associated with squamous cell carcinoma of the lung: a case report].

Abstract: We report a 59-year-old man who developed dysesthesia in all extremities with severe loss of deep sensation over three months. A radiating radicular pain was also noted in the extremities. The nerve conduction study barely elicited sensory nerve action potentials both in the median and in the sural nerve. An extensive search for anti-neuronal antibodies including anti-Hu and anti-CV2 antibody was negetive. The biopsy specimen of an enlarged tracheobronchial lymph node revealed squamous cell carcinoma. The subsequent chemotherapy and radiation therapy for the neoplasm improved the radicular pain and the deep sensation to a moderate extent, leading to the diagnosis of paraneoplastic subacute sensory neuropathy (SSN). In general, cases with paraneoplastic SSN are associated mostly with small cell lung cancer, and quite rarely with squamous cell lung cancer. The early detection and the treatment of the primary tumor are crucial in a patient with subacute progression of sensory-dominant neuropathy.