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RESUMEN La enfermedad de Parkinson es una entidad neurodegenerativa con alteraciones principalmente extrapiramidales, del sistema nervioso autónomo y, en etapas finales, alteraciones cognitivas y neuropsiquiátricas. Desde 1976 se reconocen además síntomas neurosensoriales dependientes del sistema nervioso periférico, presentes en casi la mitad de los pacientes y que preceden incluso a los síntomas motores. Los síntomas sensitivos fueron inicialmente atribuidos a distonías o a niveles indebidos de levodopa en ausencia de medicación apropiada; sin embargo, estudios recientes sugieren una etiología intrínseca a la propia enfermedad. Se reconocen tres formas de presentación: polineuropatía crónica (la más común), aguda y subaguda. La clínica de la polineuropatía es concurrente con la de la enfermedad de Parkinson y el diagnóstico es básicamente clínico, aun cuando se cuenta con el apoyo de la electromiografía y de la velocidad de conducción nerviosa, cuya normalidad no descartaría a esta entidad. El manejo actual está basado en la presunción etiológica, por lo que la reposición de vitaminas como la B12, B1 y folatos puede considerarse como parte del mismo. La etiología, el pronóstico y la fisiopatología son aún temas de debate y requieren, por lo tanto, más investigaciones. SUMMARY Parkinson´s disease is a neurodegenerative entity with predominant extrapyramidal and dysautonomic features and, in the final stages, cognitive and neuropsychiatric manifestations. Since 1976, the involvement of the peripheric nervous system has been recognized, mainly through neurosensorial symptoms affecting almost half of the Parkinson patients, even before the onset of motor symptoms. Initially, these symptoms were attributed to dystonia or the use of levodopa in the absence od medications; however, recent studies suggest an intrinsic etiology in idiopathic Parkinson´s disease. Chronic (the most frequently described), subacute and acute polyneuropathy are the main clinical forms. The sensory symptoms coexist with those of Parkinson´s disease. The diagnosis is essentially based on a clinical examination supported by electromyography and tests of neuro-conduction velocity whose normality does not rule out this entity, however. Its management depends on the etiological assumptions, and includes the eventual use of vitamin B12, B1 and folates replacement therapy. The etiology, the prognosis and the physiopathology are still topics of debate whose clarification requires further research.

The term ‘porphyria’ comes from the Greek ‘porphyra’. It refers to a heterogeneous group of metabolic disorders caused by the enzymatic deficiency in the biosynthesis of the heme group. Acute intermittent porphyria is caused by a deficiency of the porphobilinogen deaminase enzyme. A 40-year-old woman presented with abdominal pain for ten days (which required laparotomy that evidenced no surgical pathology), severe hydroelectrolytic disorder due to hyponatremia and resistant hypokalemia, persistent tachycardia and hypertension. Seven days later, she developed acute flabby quadriparesis and presented a single generalized tonic-clonic convulsive crisis. Neurophysiological studies supported mixed axonal polyneuropathy and urine results of porphobilinogen and porphyrins were elevated. After acute intermittent porphyria was diagnosed, hemin was administered, which stabilized the patient’s clinical signs and normalized the porphobilinogen. The prevalence of this entity is 1 in 2,000 people. It is an autosomal dominant disease, which affects mainly women between 20 and 40 years of age. This entity manifests with neurological and visceral symptoms. Management consists of hematin and dextrose administration avoiding hypotonic solutions because of the risk of exacerbating hyponatremia.El término 'porfiria' proviene del griego 'porphyra' y alude a un grupo heterogéneo de trastornos metabólicos causados por una deficiencia enzimática en la biosíntesis del grupo hemo. La causa de la porfiria intermitente aguda es la deficiencia de la enzima deaminasa del porfobilinógeno. Se presenta el caso de una mujer de 40 años que presentó dolor abdominal de 10 días de evolución, trastorno hidroelectrolítico grave debido a hiponatremia e hipopotasemia, taquicardia e hipertensión arterial sistémica persistentes, por lo cual fue sometida a una laparotomía en la que no se encontró ninguna afección de origen quirúrgico, A los siete días del examen inicial, la paciente desarrolló cuadriparesia flácida aguda y presentó una crisis convulsiva tónico-clónica generalizada. Los estudios neurofisiológicos evidenciaron una polineuropatía axonal mixta, y los valores de porfobilinógeno y porfirinas en orina eran elevados. Tras diagnosticarse porfiria intermitente aguda, esta se trató con hemina, lo que estabilizó los signos clínicos y normalizó el porfobilinógeno. La prevalencia de esta enfermedad es de 1 en 2.000 personas. Tiene un patrón de herencia autosómico dominante y se manifiesta principalmente en mujeres con edades entre los 20 y los 40 años. La enfermedad cursa con síntomas neurológicos y viscerales, y se trata con la administración de hemina y dextrosa, evitando las soluciones hipotónicas por el riesgo de exacerbar la hiponatremia.

Introducción: el síndrome de Guillain-Barré (SGB) es una polineurorradiculopatía desmielinizante aguda inmunomediada. El síntoma principal es la debilidad muscular. El objetivo del presente estudio es describir las características del episodio agudo, las secuelas neurológicas a largo plazo y cómo estas afectan la situación laboral y las actividades de tiempo libre. Materiales y métodos: estudio realizado en dos fases: primera, descripción retrospectiva de casos; segunda, corte transversal donde se interrogaron las secuelas neurológicas y sus implicaciones. Resultados: se identificaron 63 casos de SGB con edad promedio de 50,79 años (DE 17.8), más frecuente en hombres (61,9%) y cuyo promedio de la escala Hughes en el episodio agudo fue 3,2 (DE ± 10,1). Las principales complicaciones durante la fase aguda fueron neumonía (7,94 %) y úlceras por presión (3,17 %). Se logró contactar vía telefónica a 28 pacientes, 82,1% presentó al menos un síntoma como secuela, 60,7% parestesias, 57,1% fatiga, 50% calambres y 46,4% dolor de características neuropáticas. El 32,1% tuvo cambios o retiro de su trabajo por las secuelas; 21,43% debió modificar sus actividades de tiempo libre. Conclusión: existe alta prevalencia de síntomas residuales discapacitantes después del episodio agudo de SGB con predominio de síntomas sensitivos, coincidiendo con estudios realizados en países desarrollados. Estas alteraciones ocasionan un impacto negativo en la actividad laboral y en la de tiempo libre de los pacientes.

A fraqueza muscular consiste na incapacidade de utilizar a musculatura esquelética adequadamente, com diminuição da força. É uma das razões mais comuns pelas quais os pacientes procuram cuidados médicos. O presente trabalho trata-se de um relato de caso de polineuropatia desmielinizante inflamatória crônica (CIDP), o qual foi acompanhado durante o internato em Clínica Médica no curso de Medicina. Tem como objetivo geral: descrever um caso clínico sobre fraqueza muscular acompanhado durante o internato de Clínica Médica. Este trabalho justifica-se pela necessidade de maior entendimento médico sobre esse tema por ser algo comum da prática clínica, porém com diagnósticos diferenciais dependentes da história clínica, exame físico e exames complementares. Paciente de meia idade, sexo feminino, internada com quadro de fraqueza muscular a esclarecer, iniciada investigação para polineuropatia e sendo submetida a exames complementares e iniciado tratamento empírico. A paciente apresentou melhora clínica durante internação hospitalar e seguiu posterior acompanhamento ambulatorial pela equipe multiprofissional. Durante o internamento hipóteses diagnósticas foram questionadas quanto ao quadro apresentado: Polineuropatia Desmielinizante Inflamatória Crônica, Guillain-Barré, Esclerose Múltipla, Neuromielite Óptica.

Introducción. La porfiria es un conjunto de enfermedades metabólicas que tienen como base fisiopatológica la acumulación de precursores tóxicos. Su similitud clínica con enfermedades como el síndrome de Guillain-Barre puede retrasar el diagnostico, aumentando la posibilidad de complicaciones.Presentación del caso. Paciente femenino quien presentó síntomas inespecíficos de porfiria y síndrome de Guillain-Barre. La mujer fue evaluada de manera integral y recibió tratamiento para ambas patologías, respondiendo de manera inusual.Conclusión. La respuesta farmacológica atípica encontrada y la relación causa-efecto entre ambas entidades se justifica a la luz de sus procesos fisiopatológicos y la respuesta inmune desencadenada por los mismos.

The Paraguayan War ended 150 years ago. Back then, there were outbreaks of combatants’ limb weakness and tingling related to "palustrian cachexia", not clearly funded at the time on nutritional deficiency, the use of native flora to feed troops, and alcoholism. We report a case of a soldier with ascending paralysis, mental confusion and finally tetraplegia with preserved oculomotricity. This would probably be a case of locked-in syndrome (LIS) due to Gayet-Wernicke's encephalopathy consequent to thiamine deficiency. The role of thiamine in the peripheral or central nervous system expression was shown decades later to be related to poor diet, or use of foods containing thiaminase or thiamine antagonists, worsened by the fact that the bodily stores of thiamine are restricted, and deficits may grow fast. RESUMO A Guerra do Paraguai terminou há 150 anos. Houve surtos de fraqueza dos membros dos combatentes e parestesias relacionadas à “caquexia palustre”, não claramente fundamentados na época sobre deficiência nutricional, o uso da flora nativa para alimentar as tropas e o alcoolismo. Referimo-nos a um caso de um militar com paralisia ascendente, confusão mental e finalmente tetraplegia com oculomotricidade preservada. Esse relato seria provavelmente devido a um caso de “síndrome do encarceramento” secundário à encefalopatia de Gayet-Wernicke consequente à deficiência de tiamina. O papel da tiamina na expressão periférica ou do SNC foi previsto décadas mais tarde em relação a uma dieta pobre, ou ao uso de alimentos contendo tiamina ou antagonistas da tiamina, agravado pelo fato de que as reservas corporais de tiamina são restritas, e os déficits podem se manifestar rapidamente.

Existen variantes del síndrome de Guillain-Barre (SGB) de muy poca presentación como la debilidad distal de extremidades del SGB (DL-GBS), que muestra una debilidad limitada a las regiones distales de las extremidades (muñecas, manos, tobillos y dedos de los pies) con la fuerza muscular conservada en las regiones proximales durante el curso de la enfermedad. Se reporta el caso de una mujer de 26 años que dos semanas antes del ingreso, presenta leve déficit motor distal de las extremidades superiores y posteriormente las extremidades inferiores, asociado a diarrea. Al examen: leve cuadriparesia distal, sin hipo/arreflexia y Lasegue positivo. Los estudios de laboratorio mostraron: anemia ferropénica, las pruebas inmunológicas para virus comúnmente asociados, marcadores tumorales, pruebas toxicológicas y dosaje de Vitamina B12 fueron normales. Así mismo, el examen de líquido cefalorraquídeo no mostró alteraciones. Los estudios de RMN cerebral y columna cervical fueron normales. Los estudios de electromiografía y velocidad de conducción nerviosa (EMG-VCN) al décimo día de la enfermedad mostro una neuropatía axonal motora aguda sin denervación. Por las características benignas de su presentación y evolución hace suponer que se trata de una variante leve del SGB (DL–SGB), que difiere de las variantes normales de AMAN-SGB que generalmente son graves y de pobre pronóstico.

Journal Article; OBJECTIVES To investigate long-term safety and tolerability of capsaicin 8% patch repeat treatment in non-diabetic patients with peripheral neuropathic pain (NP). METHODS Prospective, open-label, observational study in patients with post-herpetic neuralgia, post-traumatic or post-surgical nerve injury, HIV-associated distal sensory polyneuropathy, or other peripheral NP, and average daily pain score ≥4, received ≤6 capsaicin 8% patch treatments over 52 weeks according to clinical need (retreatment at 9-12 wk intervals). Sensory testing and analgesic effectiveness were assessed using "bedside tests" and Brief Pain Inventory (question 5). RESULTS Overall, 306 patients received treatment. Treatment-emergent adverse events (TEAE) and drug-related TEAEs were reported by 252 (82.4%) and 207 (67.6%) patients. Application site pain was the most common drug-related TEAE (n=112, 36.6%); no drug-related serious TEAEs were reported. Sensory category shift analyses from baseline to end of study (EoS) in patients attending at least two sensory visits (n=278 for all tests except warm, n=277) found sensory deterioration/loss in at least one modality in 50.4% (n=140); deterioration/loss in one, two, three, four or five modalities occurred in 26.6% (n=74), 14.0% (n=39), 5.8% (n=16), 2.5% (n=7) and 1.4% (n=4). Newly emergent hyperaesthesia or allodynia was apparent in 1.1-3.6% (depending on modality) by EoS. Between 25.2 and 32.0% of patients reported improvement in a sensory modality by EoS. Average daily pain was 6.6 and 4.7 at baseline and Month 12. CONCLUSIONS Generally, capsaicin 8% patch repeat treatment over 52 weeks was well tolerated, with variable alteration in sensory function and minimal chance of complete sensory loss.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially. http://creativecommons.org/licenses/by-nc-nd/4.0. Yes

Summary Introduction: The purpose of this study was to evaluate the prevalence of peripheral polyneuropathy (PPN) in subjects with grade II and III obesity (Ob-II,III) and metabolic syndrome (MetS) but without diabetes and to investigate possible associated factors. Method: A cross-sectional study was performed in non-diabetic Ob-II,III,MetS patients using the Michigan Neuropathy Screening Instrument (MNSI) to assess the presence of PPN. Results: A total of 24 of 218 non-diabetic Ob-II,III,MetS patients had PPN. Based on univariate analysis, serum levels of LDL-cholesterol (p=0.046) were significantly associated with PPN, while serum triglycerides (p=0.118) and low HDL-cholesterol (p=0.057) showed a tendency toward this association. On a Poisson regression analysis, when the three possible associations were included, low HDL-cholesterol (p=0.047) remained independently associated. Conclusion: In non-diabetic Ob-II,III,MetS patients, PPN defined by the MNSI showed a high prevalence and was associated with low levels of HDL-cholesterol. In order to diagnose that complication, neurological evaluation should be performed in these patients.

Vasculopatia livedoide é uma doença crônica e recorrente caracterizada por livedo reticular e úlceras simétricas nos membros inferiores, que cicatrizam e deixam uma cicatriz branca atrófica ("atrophie blanche"). Envolvimento neurológico é raro e está provavelmente associado a isquemia pela trombose dos vasa nervorum. Avaliação laboratorial é indicada com o intuito de excluir causas secundárias como estados de hipercoagulabilidade, doenças autoimunes e neoplasias. Apresentamos dois pacientes com uma rara associação de vasculopatia livedoide com neuropatia periférica, enfatizando a importância de uma abordagem multidisciplinar na busca do diagnóstico correto. Livedoid vasculopathy (LV) is a chronic and recurrent disease consisting of livedo reticularis and symmetric ulcerations, primarily located on the lower extremities, which heal slowly and leave an atrophic white scar ("atrophie blanche"). Neurological involvment is rare and presumed to be secondary to the ischemia from vascular thrombosis of the vasa nervorum. Laboratory evaluation is needed to exclude secondary causes such as hypercoagulable states, autoimmune disorders and neoplasms. We present two patients with a rare association of peripheral neuropathy and LV, thereby highlighting the importance of a multidisciplinary approach to reach the correct diagnosis.

A 61-year-old woman was followed for multifocal motor neuropathy (MMN) with conduction block since young adulthood. She had bilateral clinical and neurophysiologic involvement of both median and ulnar nerves, as also the right peroneal nerve. In a recent reevaluation, we decided to complete her investigation with a brachial plexus magnetic resonance imaging (MRI) that, besides diffuse thickening of the right brachial plexus trunks, showed a round hyperintense cyst that compressed several branches distally (Figure). Brachial plexus MRI may be important [...]

Diabetic polyneuropathy can be confirmed by nerve conduction studies. The data can be analyzed in the form of a combined index instead of individual parameters. METHODS: The combined index included five parameters of nerve conduction studies commonly used for evaluation of polyneuropathies. We evaluated sensitivity in 100 diabetic patients with suspected polyneuropathy, and specificity in 200 non-diabetic patients with suspected lumbosacral radiculopathy. All results were expressed in number of standard deviations (SD). RESULTS: The sensitivity of the combined index was 81 or 74%, and specificity was 97 or 98%, using respectively -2.0 or -2.5 SD as cutoff. The range of sensitivity of the other parameters was 57-65% or 48-56%, and specificity range was 96-98% or 98-100%, using the same criteria. DISCUSSION: The combined index had higher sensitivity and equivalent specificity compared to isolated parameters.