I. Conceição

Neurophysiological evaluation of sexual dysfunction in familial amyloidotic polyneuropathy ‐ Portuguese type

Familial amyloidotic polyneuropathy (FAP) ‐ Portuguese type, is an autosomal dominant polyneuropathy related with an abnormal transthyrretin (TTR Met 30). In males, the first complaint can be sexual dysfunction. Fifteen FAP patients, mean age 37 ± 7.7 years, mean disease duration 5.2 ± 2.2 years, all males, complaining of sexual dysfunction were studied with pudendal evoked potentials (PEP), bulbocavernous reflex (BCR) and sympathetic skin response (SSR). PEP and BCR reflect the central somatosensory pathways and sacral arch functioning; SSR relates with autonomic pathways. The aims of this study were: to correlate clinical and EMG scores with somatic and autonomic fibres involvement; to evaluate the timing of somatic and autonomic nerves lesion in disease evolution. Results showed: that PEP and BCR abnormalities have a statistically significant correlation with clinical and EMG scores; abnormal SSR in the plant precede other clinical or EMG abnormalities in the present study.

Somatosensory evoked potentials in the differential diagnosis between compression and amyotrophic srinal cord literal sclerosis

Spinal cord compression (SCC) often presents a similar clinical picture to amyotrophic lateral sclerosis (ALS). An early differential diagnosis is important because SCC is a potentially treatable clinical disorder. We carried out a longitudinal study of 43 patients with an initial diagnosis of ALS, in order to ascertain the percentage of patients with SCC, and to evaluate the usefulness of somatosensory evoked potentials (SEPs) in early diagnosis. Thirty‐three patients had a final diagnosis of ALS and 8 of SCC. SEPs central conduction was abnormal in 3 ALS and 7 SCC patients, respectively (Fisher exact test, p < 0.05). We concluded that SEPs investigation is useful in the differential diagnosis between ALS and SCC patients with pure motor signs.

P854: Motor excitability measurements in familial amyloid polyneuropathy: the influence of tafamidis treatment

Question: The methylglyoxal (MGO) pathway is an offshoot of glycolysis, which converts glucose into MGO. Frequently increased in diabetic patients, MGO is highly toxic, resulting in activation of apoptosis and mitochondrial damage. Recently, MGO was found to be a causative agent for neuropathic pain. Because abnormal nerve excitability is frequently present in peripheral neuropathy and neuropathic pain, MGO can alter overall nerve excitability. The objective of the present study was to observe the chronic effects of MGO on peripheral nerve excitability in vivo. Methods: Eight adult male mice were examined. Sensory nerve multiple excitability testing (QTRAC) was performed from a tail nerve under inhalational anesthesia. The parameters were compared between at baseline and 1 week after intraperitoneal injection of MGX (0.005 ml/10g body weight) nine times (3 times weekly for 3 weeks). Results: The tested animals were all survived. The excitability parameters were similar in strength-duration time constant and threshold changes by long depolarizing current (threshold electrotonus: TE). The threshold changes by long hyperpolarizing current (TE) became smaller by MGO; (1) TEh(peak) by -70% conditioning current: -203.8±7.5 at baseline to 162.3±9.1 post-injection, P=0.003; (2) TEh(90-100ms): -94.3±4.9 at baseline to -71.7±5.5 post-injection, P=0.006. The recovery cycle showed smaller refractoriness at 2ms by MGO: 23.5±3.0 at baseline to 7.9±4.8 post-injection, P=0.01. The overall nerve excitability assessed by strength-response curves was slightly reduced after injection. Conclusions: Chronic administration of MGO changed axonal excitability in vivo. It is likely that MGO facilitated hyperpolarization-activated cation current (Ih). The upregulation of Ih may be responsible for abnormal excitability and sensory symptoms of diabetic neuropathy.

Hereditary amyloidosis related to transthyretin V30M: disease progression in treated and untreated patients

Hereditary amyloidosis related to transthyretin V30M (hATTR V30M) is a progressive length‐dependent sensorimotor axonal neuropathy. We aimed to compare the disease progression of treated [liver transplantation (LT) or tafamidis] versus untreated patients with hATTR V30M.

O puerpério como espaço educativo para o cuidado mãe e bebê

O presente relato de experiencia tem como objetivo expor e discutir as acoes de enfermagem com vistas a aumentar a competencia da mulher para o seu autocuidado no periodo puerperal e cuidado do filho recem-nascido. Na populacao alvo deste projeto, que sao as mulheres internadas no alojamento conjunto do Hospital Universitario Gaffree e Guinle, foi empregado entrevistas com foco nos conhecimentos das mulheres sobre o processo puerperal e realizado praticas de acoes educativas individuais e coletivas. Obteve-se como principais resultados maior sucesso na amamentacao, diminuicao pela instituicao de compra de leite industrializado para os recem-nascidos, diminuicao do periodo de internacao, menor retorno com problemas na amamentacao e por gravidez indesejada. Concluimos que uma assistencia individualizada que valorize os aspectos emocionais e socioculturais das puerperas, que as torne protagonistas do seu proprio cuidado reflete na sua saude, do bebe e da familia.

O2: Detection of early neuropathic abnormalities in familial amyloidotic polyneuropathy by quantitative sudomotor axon reflex test (QSART)

s of Oral Presentations Synaptic plasticity and intrinsic plasticity O1 Effects of quadripulse stimulation over medial frontal cortex on human visuomotor sequence learning T. Shimizu1, R. Hanajima1, R. Tsutsumi1, Y. Shirota2, M. Hamada1, N. Tanaka1, S. Matsuda1, Y. Terao1, Y. Ugawa3 1University of Tokyo, Neurology, Tokyo, Japan; 2University of Goettingen, Clinical Neurophysiology, Goettingen, Germany; 3Fukushima medical university, Neurology, Fukushima, Japan Questions: The pre-SMA is reported to play important roles in visuomotor sequence learning. Previous reports suggested that non-invasive brain stimulation such as rTMS or tDCs over learning related areas could modulate human motor learning process. However, the pre-SMA has not been studied in the previous studies. To study whether plastic changes in the preSMA can modulate human visuomotor sequence learning, we studied the effects of quadripulse stimulation (QPS), a new patterned rTMS technique, over preSMA on performance of the visuomotor sequential button-press task (the 2×10 task) reported by Hikosaka et al. Methods: 15 healthy volunteers participated. We applied QPS or sham stimulation over left pre-SMA for 30 minutes. QPS consisted of repeated trains of four monophasic TMS pulses separated by inter-stimulus intervals of 5 ms (QPS-5) or 50 ms (QPS-50) with an inter-train interval of 5 s. QPS-5 was reported to induce LTP in stimulated cortex, and QPS-50 LTD. After QPS, each subject performed the 2×10 task. In this task, participants asked to press 2 illuminated buttons from 16 buttons in the correct order which he must learn by trial-and-error. A total of 10 pairs were presented in a fixed order until completion. As a behavioural outcome, we counted the number of errors to complete 20 successful trials to assess the performance accuracy, and measured the button press reaction time (BP-RT): the time from stimulus onset to the first button press, and the movement time (MT): the time from the first button release to the second button press, to assess the performance speed. Results: The number of errors was larger in QPS-5 compared to sham stimulation, whereas it did not differ between QPS-50 and sham conditions. Neither MT nor BP-RT differed significantly among any stimulation conditions. Conclusion: Visuomotor sequence learning could be modulated by QPS over the pre-SMA in human. Our findings suggest that preceding regional LTP over the pre-SMA suppress visuomotor sequence learning. Changes in error number and unchanged performance speed may indicate that the pre-SMA is more associated with the acquisition of sequential procedures than the performance of sequential movements.

P221: Detection of early abnormalities in familial amyloidotic polyneuropathy by quantitative sensory testing (QST)

Discussion: In our opinion low intensity laser therapy is a new, no expensive and easy to apply encouraging treatment for CTS. References: [1] Ekim A, Armagan O, Tascioglu F, Oner C, Colak M. Effect of low level laser therapy in rheumatoid arthritis with carpal tunnel syndrome. Swiss Med Wkly. 2007; 137: 347-52. [2] Weintraub MI. Nonivasive laser neurolysis in carpal tunnel syndrome. Muscle Nerve. 1997; 20: 1029:31. [3] Irvine J, Chong SL, Amirjani NS, Chan M. Double-blind randomized controlled trial of low-level laser therapy in carpal tunnel syndrome. Muscle Nerve. 2004; 30: 182-7.

FC44.1 Longitudinal neurophysiological evaluation of olfactory mucosa mutograft in traumatic spinal cord injury

Background: Recently, in Portugal, a group started to carry out olfactory mucosa autograft (OMA) into the spinal cord of patients with chronic spinal cord injury. Aims: To describe results from longitudinal neurophysiological evaluation, in patients who underwent OMA. Population and methods: We studied 8 patients (6 males, aged from 20 to 37) with a complete spinal cord injury from C4 to D7, which occurred 1–15 years before intervention. The neurophysiological evaluation was performed before surgery, and at 4–10 weeks, 6 and 12 months after surgery. All patients underwent electromyography (nerve conduction velocities and needle EMG), Somatosensory Evoked Potentials (SEP) and Transcranial Magnetic Stimulation (TMS). The four limbs were evaluated in each patient. Results: A – Before surgery: Nerve conduction studies and needle EMG disclosed peripheral nerve lesions at entrapment sites, in 5 patients. Peroneal and ulnar nerves were the most affected. No cortical SEP or limb TMS response was recorded in any patient. B – After surgery: Lesion of the peripheral nerves remained stable. No consistent EMG voluntary recruitment could be observed below the lesion. SEPs studies detected cortical responses in 2 patients. No motor response was observed with TMS stimulation. Discussion and conclusions: Severe peripheral nerve lesions are underdiagnosed in tetraplegic-paraplegic patients and can be an important limitation for rehabilitation. SEPs can be a sensitive method to detect improvement after stem cell therapy for spinal cord injury, suggesting that the posterior cord lesion is more susceptible to recovery. We acknowledge the OMA group for performing the surgical procedure in this group of patients.