Tonic pupils: an unusual autonomic involvement in chronic inflammatory demyelinating polyneuropathy (CIDP).

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction mainly from postganglionic nerve fiber involvement. CASE REPORT: We report here an acute onset CIDP initially diagnosed as Guillain-Barré syndrome (GBS), unresponsive to treatment with intravenous immunoglobulin (IVIg), which later responded to plasmapheresis and corticoids. The patient had a markedly distal demyelination, prominent cranial nerve involvement and, interestingly, bilateral fixed dilated pupils. Despite complete clinical recovery, this neurological sign remained. CONCLUSIONS: Tonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie's pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.

Optimal E2 (reference) electrode placement in fibular motor nerve conduction studies recording from the tibialis anterior muscle.

INTRODUCTION: In this study we aimed to determine the contribution of the E2 (reference electrode) to the compound muscle action potential (CMAP) amplitude during fibular motor recording to the tibialis anterior (TA) when E2 is placed over routine referential vs. alternative sites. METHODS: The CMAP was obtained from 10 healthy subjects, using the active electrode (E1) over sites routinely used as E2 for the TA, whereas the E2 was over the contralateral knee. The same procedure was performed with the E1 over alternative E2 sites. RESULTS: Significant electrical signal was captured over routine E2 placement sites. Among the tested alternative E2 sites, the ipsilateral patella (especially its medial aspect) was the most electrically silent. DISCUSSION: Using alternative E2 sites with near isoelectric recordings can optimize near-field potential measurement in the fibular motor recording to the TA and represents a more accurate way of measuring nerve and muscle function. Muscle Nerve 59:249-253, 2019.

Motor unit number index and neurophysiological index as candidate biomarkers of presymptomatic motor neuron loss in amyotrophic lateral sclerosis.

INTRODUCTION: Our objective was to determine the utility of motor unit number index (MUNIX) and neurophysiological index (NI) as surrogate biomarkers of disease progression in limbs without clinical signs of lower motor neuron (LMN) involvement from patients with slowly progressive amyotrophic lateral sclerosis (ALS). METHODS: Patients with slowly progressive ALS and at least 1 clinically unaffected limb were prospectively enrolled. Clinical signs of LMN loss and results from hand-held dynamometer (HHD), revised ALS Functional Rating Scale (ALSFRS-R), mean-MUNIX (from 3 different muscles), and NI were longitudinally recorded. RESULTS: Eighteen patients with 43 presymptomatic muscles were evaluated. Twenty-seven muscles remained clinically unaffected during study, with stable ALSFRS-R subscores and HHD measures. However, a significant decline in mean-MUNIX and NI was detected. DISCUSSION: Mean-MUNIX and NI were more sensitive than clinical measures at detecting LMN loss in presymptomatic limbs from patients with slowly progressive ALS. Therefore, these electrophysiological biomarkers should be included in early study phases as meaningful outcome measures. Muscle Nerve 58: 204-212, 2018.

Expression of praxis induction on cortical excitability in juvenile myoclonic epilepsy.

OBJECTIVE: This study aimed to evaluate the effects of praxis induction on sensorimotor cortical and transcallosal excitability in juvenile myoclonic epilepsy (JME). METHODS: A total of 36 subjects (18-62years) were included. The JME group was screened by video-electroencephalography neuropsychological protocol and divided into JME without praxis induction [JME-WI (n=12)], JME with praxis-induced seizures or epileptiform discharges [JME-PI (n=10)], and healthy controls (n=14). Motor and somatosensory cortical excitability and transcallosal pathways were evaluated through single-pulse transcranial magnetic stimulation (sTMS) and somatosensory evoked potentials (SEPs). RESULTS: Motor and transcallosal excitabilities tested with sTMS were not different in the motor-dominant or non-dominant hemisphere among groups. Significant differences were found in cortical SEP amplitudes in the P27 component of the non-dominant hemisphere (p=0.03, Cohen's d=0.98), N35 in the dominant hemisphere (p=0.04, Cohen's d=0.96), and P27-35 interpeak amplitude in both somatosensory cortices of the JME-PI group (p=0.03, Cohen's d=0.96; p=0.02, Cohen's d=1.05) when compared with healthy controls. Giant SEPs were observed in two (16.7%) and five (50%) patients of the JME-WI and JME-PI groups, respectively. Cortical latencies did not reveal differences. CONCLUSIONS: Praxis induction was associated with enhanced excitability in the somatosensory cortex of JME patients. SIGNIFICANCE: These findings may help clarifying the less favorable therapeutic response in the JME-PI group and indicate identifying praxis induction as an important determinant in differentiating between JME patients.

Neuroimaging features in congenital trichomegaly: the Oliver-McFarlane syndrome.

A 23-year-old woman presented to our hospital with 9 months history of progressive ataxia, visual loss since childhood due to retinitis pigmentosa and primary amenorrhea. On examination, there were also sparse scalp hair, very long and curled upwards eyelashes and short stature. Oliver-McFarlane syndrome was suspected. Brain MRI disclosed cerebellar atrophy and hyperintense signal in corticospinal tracts on FLAIR and T2-weighted images. Therefore, brain imaging must be thoroughly investigated in patients with suspected Oliver-McFarlane syndrome, in order to determinate whether cerebellar atrophy and hyperintense signal in corticospinal tracts are part of this neurological condition.

Electrophysiological evaluation of the pudendal nerve and urethral innervation in female stress urinary incontinence.

INTRODUCTION AND HYPOTHESIS: Although still a matter of debate, stress urinary incontinence (SUI) may be accompanied by damage to urethral and pelvic floor innervations, thus promoting dysfunctions of the urethral support and sphincteric closure mechanisms. The aim of this study was to analyze the pelvic floor and urethral innervations through pelvic electrophysiological tests to identify whether neurological alterations interfere with urinary continence and urethral functional activity. METHODS: This prospective study included 52 women, 33 with clinically and urodynamically proven SUI and 19 continent volunteers matched for age, height, parity, and number of vaginal deliveries by the propensity score method. The patients were divided according to the severity of urinary loss evaluated by measuring abdominal leak point pressure (ALPP). Pudendal nerve terminal motor latency (PNTML), pudendal somatosensory evoked potential (SSEP) latencies, urethral and clitoral sensory thresholds, and urethroanal reflex latency were tested. RESULTS: SUI and control subjects did not differ in PNTML, SSEP latency, and clitoral sensory thresholds. However, reduced responsiveness to urethral electrosensitivity and prolonged urethroanal reflex latency were detected in most incontinent patients. In addition, urethral electrosensitivity was altered in suspected intrinsic sphincteric dysfunction. CONCLUSIONS: Urethral afferent pathways can be altered in women with SUI and may play an important role in evoking intrinsic sphincteric dysfunction.

Paralisia facial tardia após cirurgia de schwanoma vestibular: relato de caso / Delayed facial nerve palsy after vestibular schwannoma surgery: case report

Histórico: A disfunção do nervo facial pode ocorrer imediatamenteapós a cirurgia de schwanoma vestibular ou mais raramente,horas ou dias depois da operação. Atualmente, a monitoraçãoneurofisiológica intra-operatória é uma ferramenta importante para prevenir tal lesão nas intervenções do ângulo ponto-cerebelar. Descrição do caso: Paciente masculino, 70 anos, com diminuição da acuidade auditiva esquerda e zumbidohá 3 anos. A ressonância magnética (RM) demonstrou lesão isointensa, hipercaptante de 30mm no ângulo ponto-cerebelar esquerdo, com uma pequena porção intracanalicular. O paciente foi operado por via suboccipital retrosigmóide, com monitorização intraoperatória do nervo facial por eletroneuromiografia.A remoção cirúrgica foi macroscopicamente completa. O nervo facial foi bem preservado durante o procedimento e ao término apresentava atividade elétrica normal. O paciente teve boa evolução e recebeu alta no 4º dia pós-operatório com a função do nervo facial normal (House-Brackmann grau I). No 10º dia pós-operatório iniciou com dificuldade de fechar o olho esquerdo, até paralisia completa do nervo facial (House-Brackmann grau III). O tratamento com corticóide foi realizado por 5 dias associado àfisioterapia. Em um mês houve recuperação completa da paralisia e após seis meses, o paciente estava assintomático e neurologicamenteintacto. A RM realizada no 16º dia pós-operatórioapresentou aumento de sinal em segmento do nervo facial e ausência de resíduos tumorais. O estudo imunológico no pós-operatório mostrou títulos normais de anticorpos séricos contra o vírus herpes simples e herpes zoster. Conclusões: A paralisia facial tardia é um fenômeno raro, pouco relatado e conseqüentemente pouco conhecido na prática neurocirúrgica. Considerando os altos índices de recuperação da lesão do nervo facial, não se deve indicar precocemente o tratamento cirúrgico.

Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery.

Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery / Polineuropatia axonal aguda com acometimento proximal predominante: manifestação neurológica incomum de cirurgia bariátrica

Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

A cirurgia bariátrica é freqüentemente indicada no tratamento da obesidade mórbida. Complicações previamente não relatadas têm sido associadas a essa cirurgia; dentre estas, as complicações neurológicas têm recebido destaque. Relatamos o caso de um homem de 25 anos de idade submetido a cirurgia gástrica para tratamento de obesidade mórbida que desenvolveu, dois meses após a cirurgia, fraqueza de predomínio proximal nos membros inferiores, de instalação aguda. A eletroneuromiografia demonstrou polineuropatia periférica axonal nos membros inferiores, de predomínio proximal. Após tratamento com imunoglobulina e suplementação vitamínica, apresentou rápida melhora clínica e neurofisiológica. Descrevemos as características clínicas e eletroneuromiográficas desse caso, destacando a dificuldade diagnóstica inicial, particularmente com relação ao diagnóstico diferencial com síndrome de Guillain-Barré. Discutimos a importância de acompanhamento nutricional e a eventual indicação de suplementação vitamínica de rotina nesses pacientes.