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45. Diabetic distal sensorimotor and autonomic polyneuropathy in adults with type 1 and 2 diabetes
Abstracts / Clinical Neurophysiology 127 (2016) e133–e159
treatment of chronic migraine (CM) in prophylactic drug-free patients. This study aimed to verify the efficacy of repetitive injections of onabotulinumtoxin A in CM patients with ineffective chronic poly-therapy. Patients and methods: we studied 45 CM patients (36 females and 9 males) taking multiple prophylactic/symptomatic medications who were treated with onabotulinumtoxin-A for a follow up period of 9 months. The overall dose ranged between 155 and 180 U administered in 31–36 sites following PREEMPT protocol, repeated at 12 weeks intervals. The main evaluated variables to establish drug efficacy included number of headache/migraine days and episodes, total cumulative headache hours, MIDAS and HIT-6 scores; all items were assessed at baseline and after 12–24–36 weeks during follow-up. Results: all variables showed statistically significant changes after 36-weeks time-point. We observed a responder rate (partially responder plus completely responder patients) of about 40% after the first treatment; the rate reached 90% after the third treatment. Adverse events were uncommon and did not require treatment discontinuation. Conclusions: repeated treatments with onabotulinumtoxin A in CM patients with unsatisfactory prophylactic drug treatments, was very effective in improving all variables examined, determining a measurable positive impact on quality of life. doi:10.1016/j.clinph.2015.09.052
45. Diabetic distal sensorimotor and autonomic polyneuropathy in adults with type 1 and 2 diabetes—C.M. Ministeri, S. Maccora, A.A. Modica, M.S. Aliquò, S. Giordano, F. Cardella, R. Roppolo, I. Maccora (Palermo, Italy) Emerging evidence suggests that distal symmetric sensorimotor polyneuropathy (DSPN) and diabetic autonomic neuropathy (DAN) stems from nerve dysfunction in early stage of diabetes. Neurophysiological assessment can provide an early diagnosis of such complications and should be carried out at the diagnosis and during progression of the disease. We studied 44 patients, 24 type 2 diabetes (mean age 61 years, duration 14 years, mean HBA1C 8,2%) and 20 type 1 (mean age 19 years, duration 11 years, mean HBA1 C 8,4%). All subjects complained of symptoms proper to peripheral neuropathy. Neurophysiological evaluation included Nerve Conduction Studies/Electromiography, Heart Rate Variability including time domains (mean RR-interval and RR-standard deviation), Sympathetic Skin Response (SSR). Peripheral neuropathies due to any other cause were excluded. The prevalence of DSPN, DAN and the association between DSPN and DAN was significantly higher in patients with type 2 compared with those with type 1 diabetes (P < 0.001), being the duration of disease and age significantly higher (P = 0,01) in type 2 diabetes patients. Poor glycemic control and the disease duration are some of the concurrent factors in diabetic polyneuropathy and CAN pathogenesis. Since DSPN and DAN probably share the same pathogenesis, neurophysiological follow-up should be considered as a useful tool for early diagnosis of such complications. In our opinion, however, mean duration of diabetes and the age could be an important etiologic factor of PNS. doi:10.1016/j.clinph.2015.09.053
46. Abnormal sympathetic and cardiovascular reactivity during mental stress in patients with narcolepsy and cataplexy— V. Donadio, G. Plazzi, F. Pizza, R. Liguori (Bologna, Italy) To ascertain abnormalities of sympathetic and cardiovascular reactivity during mental stress in patients with narcolepsy and cataplexy (NC). We studied 10 untreated patients with established
e143
criteria for NC and hypocretin deficiency, and 15 sex and age matched healthy subjects. Subjects underwent sitting microneurographic recording of muscle sympathetic activity (MSNA) from peroneal nerve, heart rate (HR) and blood pressure (BP) during resting state and mental stress (3-min of paced auditory serial arithmetic test -PASAT) and a cold test (two minutes immersion of a hand in ice water). The awake state was continuously monitored by an ambulatory polygraphic recorder. NC patients disclosed a tendency to show decreased MSNA and BP during resting state compared to controls although the difference was not significant. However during mental stress NC patients showed decreased MSNA, BP and HR compared to controls but no difference were found during cold test. Our data demonstrated a decreased sympathetic and cardiovascular reactivity during mental stress in NC patients suggesting a possible effect of hypocretin on autonomic reaction during mental activation as reported in animal models. doi:10.1016/j.clinph.2015.09.054
47. Autonomic dysfunction in ALS patients: An early phase study_preliminary data—F. Barbieri, L.G. Bongiovanni, G.M. Fabrizi, L. Fondrieschi, S. Monaco (Verona, Italy) Amyotrophic Lateral Sclerosis (ALS) is a fatal degenerative motoneuron disease. Since 40 years, study investigating a possible Autonomic Nervous System (ANS) involvement are reported. Although results are contradictory and studies difficult to compare, ALS appears not to be a single disease entity, but a spectrum of disorders involving mainly, but not only, motoneurons. The aim of the study was to investigate cardiovascular ANS function in ALS patients with low disability and normal respiratory function. We have included patients diagnosed with ALS according to El Escorial criteria, able to walk unaided. We excluded patients with swallowing difficulties, affected by autonomic dysfunction of other origin and taking ANS influencing drugs. We also excluded patients with respiratory insufficiency. Patients underwent cholinergic and adrenergic evaluations: SSR and standard autonomic tests during continuous monitoring of heart rate and blood pressure. We evaluated HRV, BRS and spectral analysis of HF and LF of RRI and BP. We compared patients with matched normal controls. We did not find any significant difference between groups, indicating a tendency toward a normal function of ANS in early phase of disease. We will expand our sample and possibily try a genotipic–phenotipic correlation, given the heterogeneity of ALS spectrum. doi:10.1016/j.clinph.2015.09.055
48. PoTS: Long term follow-up of an Italian cohort—F. Barbieri, L.G. Bongiovanni, L. Fondrieschi, S. Monaco (Verona, Italy) Postural Tachycardia Syndrome (PoTS) is an intermittent autonomic dysfunction characterized by orthostatic intolerance and a heart rate (HR) increase of at least 30 bpm, or a HR P 120 bpm during standing. We reviewed clinical and neurophysiological characteristics and outcome after a long term follow-up of our cohort. Patients underwent clinical assessment, standardized autonomic testing and validated questionnaires to determine the severity of autonomic symptoms. The majority of our patients are young female, without either neuropathic features or a hyperadrenergic subtype. We found normal responses to Ewing tests and an increase of LF in the power spectrum of R–R interval and diastolic BP, during head–up tilt. In the same way total peripheral resistances (TPR) were maintained, even with differences among subjects. This indicates intact, although enhanced, baroreflex function. A coexisting
treatment of chronic migraine (CM) in prophylactic drug-free patients. This study aimed to verify the efficacy of repetitive injections of onabotulinumtoxin A in CM patients with ineffective chronic poly-therapy. Patients and methods: we studied 45 CM patients (36 females and 9 males) taking multiple prophylactic/symptomatic medications who were treated with onabotulinumtoxin-A for a follow up period of 9 months. The overall dose ranged between 155 and 180 U administered in 31–36 sites following PREEMPT protocol, repeated at 12 weeks intervals. The main evaluated variables to establish drug efficacy included number of headache/migraine days and episodes, total cumulative headache hours, MIDAS and HIT-6 scores; all items were assessed at baseline and after 12–24–36 weeks during follow-up. Results: all variables showed statistically significant changes after 36-weeks time-point. We observed a responder rate (partially responder plus completely responder patients) of about 40% after the first treatment; the rate reached 90% after the third treatment. Adverse events were uncommon and did not require treatment discontinuation. Conclusions: repeated treatments with onabotulinumtoxin A in CM patients with unsatisfactory prophylactic drug treatments, was very effective in improving all variables examined, determining a measurable positive impact on quality of life. doi:10.1016/j.clinph.2015.09.052
45. Diabetic distal sensorimotor and autonomic polyneuropathy in adults with type 1 and 2 diabetes—C.M. Ministeri, S. Maccora, A.A. Modica, M.S. Aliquò, S. Giordano, F. Cardella, R. Roppolo, I. Maccora (Palermo, Italy) Emerging evidence suggests that distal symmetric sensorimotor polyneuropathy (DSPN) and diabetic autonomic neuropathy (DAN) stems from nerve dysfunction in early stage of diabetes. Neurophysiological assessment can provide an early diagnosis of such complications and should be carried out at the diagnosis and during progression of the disease. We studied 44 patients, 24 type 2 diabetes (mean age 61 years, duration 14 years, mean HBA1C 8,2%) and 20 type 1 (mean age 19 years, duration 11 years, mean HBA1 C 8,4%). All subjects complained of symptoms proper to peripheral neuropathy. Neurophysiological evaluation included Nerve Conduction Studies/Electromiography, Heart Rate Variability including time domains (mean RR-interval and RR-standard deviation), Sympathetic Skin Response (SSR). Peripheral neuropathies due to any other cause were excluded. The prevalence of DSPN, DAN and the association between DSPN and DAN was significantly higher in patients with type 2 compared with those with type 1 diabetes (P < 0.001), being the duration of disease and age significantly higher (P = 0,01) in type 2 diabetes patients. Poor glycemic control and the disease duration are some of the concurrent factors in diabetic polyneuropathy and CAN pathogenesis. Since DSPN and DAN probably share the same pathogenesis, neurophysiological follow-up should be considered as a useful tool for early diagnosis of such complications. In our opinion, however, mean duration of diabetes and the age could be an important etiologic factor of PNS. doi:10.1016/j.clinph.2015.09.053
46. Abnormal sympathetic and cardiovascular reactivity during mental stress in patients with narcolepsy and cataplexy— V. Donadio, G. Plazzi, F. Pizza, R. Liguori (Bologna, Italy) To ascertain abnormalities of sympathetic and cardiovascular reactivity during mental stress in patients with narcolepsy and cataplexy (NC). We studied 10 untreated patients with established
e143
criteria for NC and hypocretin deficiency, and 15 sex and age matched healthy subjects. Subjects underwent sitting microneurographic recording of muscle sympathetic activity (MSNA) from peroneal nerve, heart rate (HR) and blood pressure (BP) during resting state and mental stress (3-min of paced auditory serial arithmetic test -PASAT) and a cold test (two minutes immersion of a hand in ice water). The awake state was continuously monitored by an ambulatory polygraphic recorder. NC patients disclosed a tendency to show decreased MSNA and BP during resting state compared to controls although the difference was not significant. However during mental stress NC patients showed decreased MSNA, BP and HR compared to controls but no difference were found during cold test. Our data demonstrated a decreased sympathetic and cardiovascular reactivity during mental stress in NC patients suggesting a possible effect of hypocretin on autonomic reaction during mental activation as reported in animal models. doi:10.1016/j.clinph.2015.09.054
47. Autonomic dysfunction in ALS patients: An early phase study_preliminary data—F. Barbieri, L.G. Bongiovanni, G.M. Fabrizi, L. Fondrieschi, S. Monaco (Verona, Italy) Amyotrophic Lateral Sclerosis (ALS) is a fatal degenerative motoneuron disease. Since 40 years, study investigating a possible Autonomic Nervous System (ANS) involvement are reported. Although results are contradictory and studies difficult to compare, ALS appears not to be a single disease entity, but a spectrum of disorders involving mainly, but not only, motoneurons. The aim of the study was to investigate cardiovascular ANS function in ALS patients with low disability and normal respiratory function. We have included patients diagnosed with ALS according to El Escorial criteria, able to walk unaided. We excluded patients with swallowing difficulties, affected by autonomic dysfunction of other origin and taking ANS influencing drugs. We also excluded patients with respiratory insufficiency. Patients underwent cholinergic and adrenergic evaluations: SSR and standard autonomic tests during continuous monitoring of heart rate and blood pressure. We evaluated HRV, BRS and spectral analysis of HF and LF of RRI and BP. We compared patients with matched normal controls. We did not find any significant difference between groups, indicating a tendency toward a normal function of ANS in early phase of disease. We will expand our sample and possibily try a genotipic–phenotipic correlation, given the heterogeneity of ALS spectrum. doi:10.1016/j.clinph.2015.09.055
48. PoTS: Long term follow-up of an Italian cohort—F. Barbieri, L.G. Bongiovanni, L. Fondrieschi, S. Monaco (Verona, Italy) Postural Tachycardia Syndrome (PoTS) is an intermittent autonomic dysfunction characterized by orthostatic intolerance and a heart rate (HR) increase of at least 30 bpm, or a HR P 120 bpm during standing. We reviewed clinical and neurophysiological characteristics and outcome after a long term follow-up of our cohort. Patients underwent clinical assessment, standardized autonomic testing and validated questionnaires to determine the severity of autonomic symptoms. The majority of our patients are young female, without either neuropathic features or a hyperadrenergic subtype. We found normal responses to Ewing tests and an increase of LF in the power spectrum of R–R interval and diastolic BP, during head–up tilt. In the same way total peripheral resistances (TPR) were maintained, even with differences among subjects. This indicates intact, although enhanced, baroreflex function. A coexisting