66. Autonomic-sensory neuropathy onset in a patient with acute brainstem impairment – A case report

66. Autonomic-sensory neuropathy onset in a patient with acute brainstem impairment – A case report

Society Proceedings / Clinical Neurophysiology 126 (2015) e1–e28 e15 a benign purely trigeminal neuropathy, or facial-onset sensory motor neuronopat...

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Society Proceedings / Clinical Neurophysiology 126 (2015) e1–e28

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a benign purely trigeminal neuropathy, or facial-onset sensory motor neuronopathy (FOSMN), a malignant life-threatening condition. No diagnostic criteria can yet differentiate the two conditions at their onset. Nor is it clear whether the two diseases are distinct entities or share common pathophysiological mechanisms. Seeking pathophysiological and diagnostic information to distinguish these two conditions at their onset, in this neurophysiological and morphometric study we neurophysiologically assessed function in myelinated and unmyelinated fibres and histologically examined supraorbital nerve biopsy specimens with optic and electron microscopy in 13 consecutive patients with recent onset trigeminal hypoesthesia and pain. The disease course distinctly differed in the 13 patients. During a mean 10 year follow-up whereas in eight patients the disease remained relatively stable, in the other five it progressed to possibly life-threatening motor disturbances and extra-trigeminal spread. From two to six years elapsed between the first sensory symptoms and the onset of motor disorders. In patients with idiopathic trigeminal sensory neuropathy and in those with FOSMN neurophysiological and histological examination documented a neuronopathy manifesting with trigeminal nerve damage selectively affecting myelinated fibres, but sparing the Ia-fibre-mediated proprioceptive reflex. Although no clinical diagnostic criteria can distinguish the two conditions at onset, neurophysiological and nerve-biopsy findings specify that in both disorders trigeminal nerve damage manifests as a dissociated neuronopathy affecting myelinated and sparing unmyelinated fibres thus suggesting similar pathophysiological mechanisms.

64. Predictive value of neurophysiological testing and the importance of multidisciplinary approach in the pelvic floor disorders—M. Marangone TNFP, G. Squintani, M. Crovato TNFP, F. Donato, F. Leopardi, A. D’Amico, S. Romito, G. Moretto (Verona, Italy)

doi:10.1016/j.clinph.2014.10.081

doi:10.1016/j.clinph.2014.10.083

63. Multicenter protocol on the role of ultrasound in immunemediated neuropathies—I. Paolasso, L. Hobson-Webb, C. Briani, H. Tsukamoto, D. Coraci, C. Erra, G. Granata, L. Padua (Roma, Italy, Durham, NC, USA, Padova, Italy)

65. Peripheral nerve ultrasonography: Clinical use—M.C. Tozzi, M. Turri, E. Concon, T. Cavallaro, G.M. Fabrizi, S. Monaco, L. Bertolasi (Verona, Italy)

Immune-mediated neuropathies include clinically heterogeneous disorders such as Guillain–Barré syndrome (GBS) and its variants, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN), neuropathy with monoclonal gammopathy and others. High-resolution ultrasound (US) is a helpful technique for the evaluation of peripheral nerves. In previous studies on immune-mediated neuropathies, nerve US showed diffuse enlargement of cross sectional area (CSA) of peripheral nerves trunks and cervical roots and morphological alterations at conduction block site. Data are however heterogeneous, correlations with clinical history or disease severity are lacking and there are no longitudinal studies in literature. The objective of the protocol is to prospectively evaluate, through a multicenter longitudinal study, nerve US findings in patients with newly diagnosed immune-mediated neuropathies and evaluate the relationship with clinical and neurophysiological findings and changes over time (natural history) or in response to therapy. The protocol includes clinical assessment, neurophysiological examination, US examination of median, ulnar, radial, fibular, tibial, sural nerves (and brachial plexus in extended protocol) with evaluation of maximal/minimal CSA for each nerve, inter- and intra-nerve variability, US classification depending on echogenicity and fascicles enlargement. US and neurophysiological follow-up timing examinations follow different schedules in acute and chronic immune-mediated disease. We present preliminary results and feasibility of the protocol. doi:10.1016/j.clinph.2014.10.082

To evaluate the sensitivity/specificity and predictive value of neurophysiological battery in pelvic floor disorders and underlines the importance of a multidisciplinary approach. From 2008 to 2014, 72 patients came to our attention for urinary (41/72), ano-rectal (25/72), erectile (12/72) disturbances. 47 subjects with suspected pudendal neuralgia were included as controls. All subjects were submitted to neurological/perineal examination and neurophysiological battery. In selected cases, other exams (video-urodynamics, defecography, pelvic floor MRI, etc.) were performed in order to clarify the pathophysiology of the disorders. Upper and lower limits of normal values were collected and compared to patients’ data. Sensitivity, specificity, positive and negative predictive values were also calculated for each one neurophysiological test. 35/72 patients were affected by neurological disease (20 LMND, 15 UMND), while 22 presented urological/ anorectal disorders. CNEMG and sacral reflexes detected the higher sensitivity and predictive value for LMND. Multidisciplinary approach and neurophysiological testing were essential to explain the pathophysiology of symptoms in patients with a clear diagnosis. Multidisciplinary approach actually detects a key role in the management of pelvic floor disease, while a specific protocol is optimal in order to increase the sensitivity and predictive value of neurophysiological tests in perineal disorders.

Charcot–Marie–Tooth disease (CMT) is an heterogeneous group of hereditary neuropathies characterized by weakness and atrophy of the limbs, loss of sensation and absent or reduced tendon reflexes. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) causes progressive or recurrent weakness and numbness of the limbs. Both CMT and CIDP share similar nerve conduction study abnormalities (reduced amplitude of sensory and motor potentials, and reduced conduction velocity). The aim of our study was to compare the ultrasound (US) data recorded in genetic neuropathy (CMT) and inflammatory neuropathy (CIDP). We enrolled 10 patients with CMT type 1A and 10 patients with CIDP. In this study we compared left and right median nerve cross-sectional area (CSA) and echogenicity nerve in the forearm, between two groups of patients to evaluate a possible difference. Three US classes were identified based on CSA and echogenicity. For all patient included neurophysiological assessment was used to estimate a possible correlation between the nerve conduction study variables and US data. US of the nerve can be a tool of significant value in the differential diagnosis in different neuropathies. doi:10.1016/j.clinph.2014.10.084

66. Autonomic-sensory neuropathy onset in a patient with acute brainstem impairment – A case report—S. Vigneri, V. Rispoli, C. Azzini, M.L. Caniatti, M.R. Tola, V. Simioni, J.G. Capone, E. Sette, V. Tugnoli (Ferrara, Italy) Here we report on a 64-year-old Caucasian man admitted to the Neurology department due to a history of severe hypoacusia, nausea,

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stipsis, cerebellar ataxia and progressing consciousness impairment, which raised up the clinical suspicion of acute cerebellitis and suggested the introduction of oral corticosteroids. At day 7, a mild improvement of cerebellar symptoms was followed by the onset of orthostatic hypotension, four limb sensory impairment and areflexia. The spinal tap displayed a mild protein increase (78 mg/dl) and hightiter of anti-Hu (ANNA-1) antibodies in CSF. The same antibodies were also found after blood testing. Nerve conduction velocities (NCV) of the four limbs showed a picture of severe peripheral neuropathy with predominant sensory and axonal features, absence of sympathetic skin response. No sensory evoked potentials were detectable either at upper and lower limbs, while cranial district reflexes and NCV were not affected. Autonomic tests confirmed severe orthostatic hypotension with mild parasympathetic involvement. A PET study highlighted a hypercaptation in the right paratracheal region, which a bronchoscopy with transbronchial biopsy identified as a possible lung cancer. Therefore, the patient was diagnosed with dysimmune rhombencephalitis and polyneuropathy in a likely paraneoplastic syndrome.

only poorly understood. We evaluated the effects of cerebellar transcranial direct current stimulation (c-tDCS) on pain perception by assessing changes in laser evoked potentials (LEPs) parameters (perceptive threshold, N1–N2/P2 amplitudes and latencies) by stimulating the left hand. Materials and Methods: Twelve healthy subjects were enrolled and studied before and after anodal, cathodal and sham tDCS (20’, 2.0 mA). LEPs were obtained using a neodymium:yttrium–aluminium–perovskite (Nd:YAP) laser. While cathodal c-tDCS improves amplitudes (N1: F(2,66) = 23.3, p < 0.0001; N2/P2: F(2,66) = 19.1, p < 0.0001) and decreases LEPs latencies (N1: F(2,66) = 7.7, p = 0.001; N2/P2: F(2,66) = 5.4, p = 0.007) compared with sham condition, anodal c-tDCS elicits opposite effects (p < 0.001 for all the comparisons). Anodal polarization dampens perceptive threshold, while the cathodal stimulation increases it (p < 0.001). The main finding of our study is that cerebellar direct current polarization is able to modulate pain perception in humans. As c-tDCS was effective on N1 and N2/P2 components, we speculate that cerebellum is engaged in pain processing dynamically modulating the activity of both secondary somatosensory and cingulate cortices.

doi:10.1016/j.clinph.2014.10.085 doi:10.1016/j.clinph.2014.10.087

67. Does the epidermal nerve fibre density measured by skin biopsy in patients with peripheral neuropathies correlate with neuropathic pain?—A. Biasiotta, C. Leone, S. La Cesa, A. Pepe, E. Galosi, S. Piroso, G. Di Stefano, C. Giordano, G. Cruccu, A. Truini (Roma, Italy) The different neuropathic pain types (e.g. ongoing burning pain and allodynia) are frequent and disabling complaints in patients with peripheral neuropathies. Although the reference standard technique for diagnosing painful small fibre neuropathies is nerve fibre density assessment by skin biopsy, the relationship between the epidermal nerve fibre (ENF) density and neuropathic pain is still unclear. In a clinical and skin biopsy study designed to investigate whether changes in ENF density are directly related to pain, we enrolled 139 consecutive patients with distal symmetric peripheral neuropathy. All patients underwent clinical examination. The Neuropathic Pain Symptom Inventory was used to distinguish the different neuropathic pain types. A skin biopsy was conducted and ENFs were immunostained with the antiprotein gene product 9.5 and their linear density was quantified with bright-field microscopy. No difference was found in ENF density between patients with and without neuropathic pain, nor between patients with and without ongoing burning pain. Conversely, ENF density was higher in patients with provoked pains (including mechanical dynamic allodynia) than in those without. The variable association between ENF density and symptoms of neuropathic pain supports the idea that neuropathic pain symptoms arise through distinct underlying mechanisms. The lack of relationship between ongoing burning pain and ENF density suggests that this type of pain reflects factors other than loss of nociceptive afferents. The association between ENF density and provoked pain (including mechanical dynamic allodynia) suggests that this type of pain might be mediated by spared and sensitised nociceptive afferents. doi:10.1016/j.clinph.2014.10.086

68. Cerebellar transcranial direct current stimulation modulates pain perception in humans: A laser evoked potentials (LEPS) study—T. Bocci, B. Vannini, A. Torzini, E. Giorli, R. Ferrucci, G. Carli, A. Priori, F. Sartucci (Siena, Italy, Pisa, Italy, Milano, Italy) Cerebellum is involved in a wide number of integrative functions, most of them unknown. Particularly, its role in pain processing has

69. Reduced visual cortical reactivity in migraineurs with a positive family history of migraine—G. Coppola, M. Bracaglia, D. Di Lenola, G. Di Ciaccia, C. Di Lorenzo, M. Serrao, V. Parisi, F. Pierelli (Roma, Italy) In migraine, the genetic load can be seen as determining, on the one hand, a critical threshold for the disease development, and on the other hand, it may be responsible for interictal nervous system dysfunction. We were aimed at verifying whether a family history of migraine might influence migraineurs’ cortical abnormal information processing. We retrospectively collected 109 migraine patients from those reviewed who had visual evoked potentials (VEPs) recordings (6 blocks of 100 sweeps, 15 min of arc cheques, 3.1 repetition rate) and information about parental history of migraine. Neurophysiological data were compared with those of 42 healthy volunteers (HV) without family history of migraine. We recruited 109 migraineurs, 85 with and 24 without a positive family history of migraine. Patients who had one parent affected (mother or father) had significantly lower N75-P100 VEP amplitude blocks overall than those had no parents affected, the latter resulting not different from HV. Lack of VEP N75-P100 amplitude habituation was found in overall migraineurs compared with HV, irrespectively of whether they had a parent affected or not. These findings suggest that familial occurrence of migraine may predispose to a general reduced cortical reactivity to visual stimulation. doi:10.1016/j.clinph.2014.10.088

70. Correlation between abnormal brain excitability, anger management and anxiety in migraine children—E. Iacovelli, S. Tarantino, S. Pro, C. Vollono, A. Capuano, R. Torriero, F. Vigevano, M. Valeriani (Roma, Italy) To analyze the possible correlation between abnormal brain excitability and psychological factors in migraine children. We studied 12 migraine children. P300 response was recorded in three successive blocks to test EP habituation. Psychological profile was assessed by Picture Frustration Study test for anger management (PFS) and Psychiatric scales for self-administraion for youths and adolescents (SAFA-A scale for anxiety). In migraineurs, all the EP components (N1, P2, and P300) showed a reduced habituation, as