HbA1c and the risk of developing peripheral neuropathy in childhood-onset type 1 diabetes: A follow-up study over 3 decades.

AIMS: We have evaluated long-term weighted mean HbA1c (wHbA1c), HbA1c variability, diabetes duration, and lipid profiles in relation to the development of diabetic peripheral neuropathy (DPN), nephropathy, and retinopathy in childhood-onset type 1 diabetes. MATERIALS AND METHODS: In a longitudinal cohort study, 49 patients (21 women) with childhood-onset type 1 diabetes were investigated with neurophysiological measurements, blood tests, and clinical examinations after a diabetes duration of 7.7 (±3.3) years (baseline) and followed with repeated examinations for 30.6 (±5.2) years. We calculated wHbA1c by integrating the area under all HbA1c values since the diabetes diagnosis. Lipid profiles were analysed in relation to the presence of DPN. Long-term fluctuations of HbA1c variability were computed as the standard deviation of all HbA1c measurements. Data regarding the presence of other diabetes complications were retrieved from medical records. RESULTS: In this follow-up study, 51% (25/49) of the patients fulfilled electrophysiological criteria for DPN. In nerve conduction studies, there was a deterioration in the amplitudes and conduction velocities for the median, peroneal, and sural nerves over time. Patients with DPN had a longer duration of diabetes, higher wHbA1c, and increased HbA1c variability. The lowest wHbA1c value associated with the development of DPN was 62 mmol/mol (7.8%). The presence of albuminuria and retinopathy was positively correlated with the presence of neuropathy. CONCLUSIONS: More than half of the patients had developed DPN after 30 years. None of the patients who developed DPN had a wHbA1c of less than 62 mmol/mol (7.8%).

Prevalence of discomplete sensorimotor spinal cord injury as evidenced by neurophysiological methods: A cross-sectional study.

OBJECTIVES: To assess the prevalence of residual trans-lesion connectivity in persons with chronic clinically complete spinal cord injury (discompleteness) by neurophysiological methods. PARTICIPANTS: A total of 23 adults with chronic sensorimotor complete spinal cord injury, identified through regional registries the regional spinal cord registry of Östergötland, Sweden. METHODS: Diagnosis of clinically complete spinal cord injury was verified by standardized neurological examination. Then, a neurophysiological examination was performed, comprising electroneurography, electromyography, sympathetic skin response and evoked potentials (sensory, laser and motor). Based on this assessment, a composite outcome measure, indicating either strong, possible or no evidence of discomplete spinal cord injury, was formed. RESULTS: Strong neurophysiological evidence of discomplete spinal cord injury was found in 17% (4/23) of participants. If also accepting "possible evidence", the discomplete group comprised 39% (9/23). The remaining 61% showed no neurophysiological evidence of discompleteness. However, if also counting reports of subjective sensation elicited during neurophysiological testing in the absence of objective findings, 52% (12/23) showed indication of discomplete spinal cord injury. CONCLUSION: Evidence of discomplete spinal cord injury can be demonstrated using standard neurophysiological techniques in a substantial subset of individuals with clinically complete spinal cord injury. This study adds to the evidence base indicating the potential of various modes of cross-lesional sensorimotor functional restoration in some cases of chronic clinically complete spinal cord injury.

Acute motor axonal neuropathy associated with anal carcinoma: Paraneoplastic neurological syndrome or coincidence?

AIM: Assessment of the association of an acute motor axonal neuropathy with a squamous cell anal carcinoma. BACKGROUND: Paraneoplastic neurologic syndromes are not a direct consequence of neither primary tumor nor its metastasis. They often parallel the course of the malignancy but may be the presenting sign of an occult cancer. Sometimes it is very difficult to distinguish if it is a paraneoplastic syndrome or just a coincidence. MATERIALS AND METHODS: We report a 60-year-old man that presented with an acute motor deficit of the four limbs. Clinical examination found a pure and severe motor deficit in the four limbs. No sensory abnormality was found and all motor nerves were unexcitable. Electromyography suggested the diagnosis of acute motor axonal neuropathy (AMAN). Four months after developing the AMAN, blood in the stool revealed anal carcinoma. The patient was treated with concurrent chemoradiotherapy. Radiation was given to the tumor and to the pelvis, including inguinal nodes, over a five-week period plus fluorouracil and mitomycin. We investigated the presence of antiganglioside antibodies as studies suggest that carcinomas can express antigens shared with Schwann cells. RESULTS: Anti-GM1 IgG antibodies were detected by an enzyme-linked immunosorbent assay method. Other antibodies, including antinuclear nucleoprotein antibody (anti-Hu), anti-Tr, anti-Ri, anti-CV2, anti-amphiphysin and anti-Yo, were negative. Clinical improvement of the motor state was observed at the fourth week of oncologic treatment. CONCLUSION: The presence of anti-GM1 IgG antibodies and the clinical improvement of the motor state after concurrent chemoradiotherapy lead us to believe there is an association between anal carcinoma and this severe impairment.