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92. Neurophysiology and ultrasound in diagnosis of isolated peripheral nerve tumors. Literature revision and personal experience
Society Proceedings / Clinical Neurophysiology 126 (2015) e1–e28
89. Segmental muscular neurogenic hypertrophy: Two case reports with different site of lesion—R. Caramelli, V. Schiavone, A. Cassardo, A. Amantini, G. de Scisciolo (Firenze, Italy) The usual semiotic sign of motor neuron damage is hypotrophy of target muscle. However, in rare cases, a motor neuron damage results in hypertrophy: this is principally due to a chronic triggering of spontaneous activity in affected motor units, with continuous or subcontinuous activation of target muscular fibers. Muscular neurogenic hypertrophy was reported during radiculopathy, mononeuropathy, focal myelopathy, tethered cord syndrome, chronic inflammatory demyelinating polyneuropathy. In this report we present classification of muscular neurogenic hypertrophy, based on topography, etiology and type of spontaneous activity possibly observed. We present also two cases: the first one due to spondyloarthrosis associated with T11–T12 discopathy, the second one due to ischiatic nerve neuropathy related to proximal femur fracture. doi:10.1016/j.clinph.2014.10.108
90. Strength and respiratory evaluation in ALS: A follow up study—M. Ceccanti, C. Cambieri, G. Capua, D. Lopergolo, V. Frasca, M. Gabriele, M. Inghilleri (Roma, Italy) Amyotrophic lateral sclerosis is a degenerative disease involving upper and lower motor neurons, thus leading to progressive spasticity and weakness. Purpose of this study is to relate reduction in cMAP amplitude to functional impairment in ALS patients. We recruited 156 patients, 50 with bulbar, 106 with spinal onset. Patients were periodically visited every 3 month; medial plantar, ulnar and frenic nerves were examined in each patient. ALSFRS-R scale and MRC scale for strength were submitted; forced vital capacity and oxygen saturation were measured. In spinal onset patients, plantar medial reduction correlated to MRC for ipsilateral limb decrease between visit 1–2 (p = 0.034) and 1–3 (p = 0.006). Reduction in plantar medial cMAP in visit 1–3 and 1–4 correlated to FVC impairment in the same period (p = 0.037 and 0.008, respectively). Frenic cMAP decline between visit 1 and 4 was associated to ALSFRS impairment in respiratory items (p = 0.033). In bulbar onset, plantar medial reduction correlated to MRC for ipsilateral limb decrease between visit 1–2 (p = 0.002) and 1–3 (p = 0.044). Ulnar reduction between visit 1 and 4 was related to MRC for ipsilateral limb (p = 0.002), ALSFRS spinal (p = 0.032) and total (p = 0.028) score reduction. All these data are demonstrating a key-role of cMAP examination in monitoring strength and respiratory failure in ALS patients. doi:10.1016/j.clinph.2014.10.109
91. Neurophysiological evaluation of spinal excitability in patients affected by primitive restless legs syndrome—P. Congiu, G. Milioli, G. Gioi, P. Tacconi, M.L. Fantini, M. Figorilli, F. Marrosu, L. Parrino, M. Puligheddu (Cagliari, Italy) The aim of our study is to explore the spinal excitability through the electrophysiological evaluation of patients affected by primitives Restless Legs Syndrome (RLS). 15 women affected by primitive RLS were compared with age-matched healthy females. All subjects underwent ENG evaluation: nerve conduction studies (NCS), F waves parameters (minimum, mean and maximum latency, duration, amplitude, persistency and the ratio between F wave duration (FWD) and the duration of the corresponding compound muscle action potential (FWD/CMAPD), and the Cutaneous Silent Period parameters (latency, duration and the ratio between duration at
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low to upper extremities). None of the subjects included in our study showed alterations in the NCS. Compared to the control group, RLS patients showed significantly higher values of the FWD for both ulnar (p < 0.05) and tibial (p < 0.01) nerves and for the FWD/CMAPD ratio (p < 0.001). The results of our study indicate a widespread spinal motoneuronaI hyper-excitability that could be mainly due to an altered modulation within the interneuronal system. Presently, RLS diagnosis is based exclusively on clinical criteria. The FWD/ CMAPD ratio can help to shed light on the pathogenesis of RLS, and can represent an objective, easily obtainable, diagnostic tool especially in cases of evening lower leg discomfort of unclear interpretation. doi:10.1016/j.clinph.2014.10.110
92. Neurophysiology and ultrasound in diagnosis of isolated peripheral nerve tumors. Literature revision and personal experience—D. Coraci, V. Santilli, C. Erra, P. De Franco, L. Padua (Roma, Italy, Milano, Italy) Our work shows the association of neurophysiology and ultrasound in the assessment of peripheral nerve tumors. In these cases, neurophysiologic examination presents an important role to define the possible lack of function of the nerve. Ultrasonography allows us to visualize the peripheral nerves and underline the morphologic changes in nerve structure. We present a literature revision about ultrasound in peripheral nerve tumor study and a collection of 10 cases where the combined use of clinic, neurophysiology and ultrasound guided the diagnostic process of nerve tumors. All the patients presented a clinic pattern of nerve involvement and everyone was evaluated with neurophysiology which confirmed a mononeuropathy, but without the possibility of a sure diagnosis. The following ultrasound evaluation allowed to visualize a focal lesion, compatible with a nerve tumor. These cases show that neurophysiology and ultrasound together contribute in diagnosis of peripheral nerve tumors. Ultrasonography is not able to certainly define the type of tumor, but it gives suggestions about discrimination between schwannoma and neurofibroma. Nerve ultrasound is useful in the assessment of peripheral nerve tumors. doi:10.1016/j.clinph.2014.10.111
93. CANOMAD: Clinical and neurophysiological findings in two cases—E. Del Sordo, S. Casali, F. Ginanneschi, L. Insana, G. Capoccitti, C. Cardinali, D. Battista, A. Borgheresi, M. Cincotta, W. Borsini, F. Giannini (Firenze, Italy, Siena, Italy) The acronym CANOMAD (Chronic Ataxic Neuropathy, Ophthalmoplegia, IgM paraproteinemia, cold Agglutinin and Disialosyl antibodies) refers a rare immune-mediated peripheral neuropathy, whose clinical spectrum has still to be fully delineated. Herein we report the clinical and neurophysiological features of two CANOMAD patients. A 52-year-old man suffering from distal sensory symptoms and ataxia had received a first diagnosis of chronic inflammatory prominent-sensory polyneuropathy 11 years ago. High-dose i.v. steroid treatment resulted in paradoxical severe worsening with ophthalmoplegia and ambulation loss. Based on immunological and clinical–neurophysiological revaluation a diagnosis of CANOMAD was made. Since then IVIg periodic infusions had allowed good clinical remission up to-day, although minor degree of motor impairment and ataxia relapsed during last years. This 62-year-old man had a 5-year history of mild lower limb ataxia. More recently, a fluctuating bilateral ptosis was seen.
89. Segmental muscular neurogenic hypertrophy: Two case reports with different site of lesion—R. Caramelli, V. Schiavone, A. Cassardo, A. Amantini, G. de Scisciolo (Firenze, Italy) The usual semiotic sign of motor neuron damage is hypotrophy of target muscle. However, in rare cases, a motor neuron damage results in hypertrophy: this is principally due to a chronic triggering of spontaneous activity in affected motor units, with continuous or subcontinuous activation of target muscular fibers. Muscular neurogenic hypertrophy was reported during radiculopathy, mononeuropathy, focal myelopathy, tethered cord syndrome, chronic inflammatory demyelinating polyneuropathy. In this report we present classification of muscular neurogenic hypertrophy, based on topography, etiology and type of spontaneous activity possibly observed. We present also two cases: the first one due to spondyloarthrosis associated with T11–T12 discopathy, the second one due to ischiatic nerve neuropathy related to proximal femur fracture. doi:10.1016/j.clinph.2014.10.108
90. Strength and respiratory evaluation in ALS: A follow up study—M. Ceccanti, C. Cambieri, G. Capua, D. Lopergolo, V. Frasca, M. Gabriele, M. Inghilleri (Roma, Italy) Amyotrophic lateral sclerosis is a degenerative disease involving upper and lower motor neurons, thus leading to progressive spasticity and weakness. Purpose of this study is to relate reduction in cMAP amplitude to functional impairment in ALS patients. We recruited 156 patients, 50 with bulbar, 106 with spinal onset. Patients were periodically visited every 3 month; medial plantar, ulnar and frenic nerves were examined in each patient. ALSFRS-R scale and MRC scale for strength were submitted; forced vital capacity and oxygen saturation were measured. In spinal onset patients, plantar medial reduction correlated to MRC for ipsilateral limb decrease between visit 1–2 (p = 0.034) and 1–3 (p = 0.006). Reduction in plantar medial cMAP in visit 1–3 and 1–4 correlated to FVC impairment in the same period (p = 0.037 and 0.008, respectively). Frenic cMAP decline between visit 1 and 4 was associated to ALSFRS impairment in respiratory items (p = 0.033). In bulbar onset, plantar medial reduction correlated to MRC for ipsilateral limb decrease between visit 1–2 (p = 0.002) and 1–3 (p = 0.044). Ulnar reduction between visit 1 and 4 was related to MRC for ipsilateral limb (p = 0.002), ALSFRS spinal (p = 0.032) and total (p = 0.028) score reduction. All these data are demonstrating a key-role of cMAP examination in monitoring strength and respiratory failure in ALS patients. doi:10.1016/j.clinph.2014.10.109
91. Neurophysiological evaluation of spinal excitability in patients affected by primitive restless legs syndrome—P. Congiu, G. Milioli, G. Gioi, P. Tacconi, M.L. Fantini, M. Figorilli, F. Marrosu, L. Parrino, M. Puligheddu (Cagliari, Italy) The aim of our study is to explore the spinal excitability through the electrophysiological evaluation of patients affected by primitives Restless Legs Syndrome (RLS). 15 women affected by primitive RLS were compared with age-matched healthy females. All subjects underwent ENG evaluation: nerve conduction studies (NCS), F waves parameters (minimum, mean and maximum latency, duration, amplitude, persistency and the ratio between F wave duration (FWD) and the duration of the corresponding compound muscle action potential (FWD/CMAPD), and the Cutaneous Silent Period parameters (latency, duration and the ratio between duration at
e21
low to upper extremities). None of the subjects included in our study showed alterations in the NCS. Compared to the control group, RLS patients showed significantly higher values of the FWD for both ulnar (p < 0.05) and tibial (p < 0.01) nerves and for the FWD/CMAPD ratio (p < 0.001). The results of our study indicate a widespread spinal motoneuronaI hyper-excitability that could be mainly due to an altered modulation within the interneuronal system. Presently, RLS diagnosis is based exclusively on clinical criteria. The FWD/ CMAPD ratio can help to shed light on the pathogenesis of RLS, and can represent an objective, easily obtainable, diagnostic tool especially in cases of evening lower leg discomfort of unclear interpretation. doi:10.1016/j.clinph.2014.10.110
92. Neurophysiology and ultrasound in diagnosis of isolated peripheral nerve tumors. Literature revision and personal experience—D. Coraci, V. Santilli, C. Erra, P. De Franco, L. Padua (Roma, Italy, Milano, Italy) Our work shows the association of neurophysiology and ultrasound in the assessment of peripheral nerve tumors. In these cases, neurophysiologic examination presents an important role to define the possible lack of function of the nerve. Ultrasonography allows us to visualize the peripheral nerves and underline the morphologic changes in nerve structure. We present a literature revision about ultrasound in peripheral nerve tumor study and a collection of 10 cases where the combined use of clinic, neurophysiology and ultrasound guided the diagnostic process of nerve tumors. All the patients presented a clinic pattern of nerve involvement and everyone was evaluated with neurophysiology which confirmed a mononeuropathy, but without the possibility of a sure diagnosis. The following ultrasound evaluation allowed to visualize a focal lesion, compatible with a nerve tumor. These cases show that neurophysiology and ultrasound together contribute in diagnosis of peripheral nerve tumors. Ultrasonography is not able to certainly define the type of tumor, but it gives suggestions about discrimination between schwannoma and neurofibroma. Nerve ultrasound is useful in the assessment of peripheral nerve tumors. doi:10.1016/j.clinph.2014.10.111
93. CANOMAD: Clinical and neurophysiological findings in two cases—E. Del Sordo, S. Casali, F. Ginanneschi, L. Insana, G. Capoccitti, C. Cardinali, D. Battista, A. Borgheresi, M. Cincotta, W. Borsini, F. Giannini (Firenze, Italy, Siena, Italy) The acronym CANOMAD (Chronic Ataxic Neuropathy, Ophthalmoplegia, IgM paraproteinemia, cold Agglutinin and Disialosyl antibodies) refers a rare immune-mediated peripheral neuropathy, whose clinical spectrum has still to be fully delineated. Herein we report the clinical and neurophysiological features of two CANOMAD patients. A 52-year-old man suffering from distal sensory symptoms and ataxia had received a first diagnosis of chronic inflammatory prominent-sensory polyneuropathy 11 years ago. High-dose i.v. steroid treatment resulted in paradoxical severe worsening with ophthalmoplegia and ambulation loss. Based on immunological and clinical–neurophysiological revaluation a diagnosis of CANOMAD was made. Since then IVIg periodic infusions had allowed good clinical remission up to-day, although minor degree of motor impairment and ataxia relapsed during last years. This 62-year-old man had a 5-year history of mild lower limb ataxia. More recently, a fluctuating bilateral ptosis was seen.