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Introduction: Myeloneuropathy is a diagnosis ascribed to disorders that concomitantly affect the spinal cord and peripheral nerves. Recognizing this syndrome may sometimes be arduous, even for the most consummate clinicians, because symptomatology can mimic either spinal cord or peripheral nerve disease. Besides, examination findings suggest a predominantly myelopathic or neuropathic picture. This article reports a rendezvous of rare cases of clinically diagnosed myeloneuropathy with different etiological backgrounds and therapeutic responses. Methods: Eleven cases of non-compressive myeloneuropathy were admitted to the Department of General Medicine of Burdwan Medical College and Hospital, Burdwan, West Bengal, India, between May 2018 and May 2022. Results: We report the cases of 11 patients (6 men and 5 women) who presented with myeloneuropathy of different etiologies (vitamin B12, copper, and vitamin E deficiencies, organophosphate poisoning, chronic alcohol abuse, illicit substances abuse, anti-thyroid peroxidase/anti-thyroglobulin antibody-related neurologic disorder responsive to steroids, Sjögren syndrome, chikungunya infection, paraneoplastic, and hereditary). Conclusion: Meticulous historical analysis, careful clinical examination, and apposite utilization and interpretation of biochemical, electrophysiological, and neuroimaging findings are sine-qua-non for an accurate and consistent approach to evaluating a suspected case of myeloneuropathy, facilitating early treatment and recovery. Differential identification of these disorders needs an in-depth perception of the mode of onset of symptoms, the course of progression of the disease, the pattern of myelopathic/neuropathic findings, and recognition of other neurological or systemic manifestations. For untroubled understanding, etiologies of myeloneuropathies should be subdivided into a few broad categories, e.g., metabolic (nutritional), toxic (toxin-induced), infectious, inflammatory (immune-mediated), paraneoplastic, and hereditary disorders.
Introducción: La mieloneuropatía es un síndrome que afecta concomitantemente la médula espinal y los nervios periféricos. Reconocerlo a veces puede ser arduo, incluso para los médicos más experimentados, porque la sintomatología puede simular una enfermedad de la médula espinal o de los nervios periféricos. Además, los hallazgos del examen sugieren un cuadro predominantemente mielopático o neuropático. Este artículo describe una serie de casos raros de mieloneuropatía de distintas causas y con respuestas terapéuticas distintas. Métodos: Once casos de mieloneuropatía no compresiva fueron ingresados en el Departamento de Medicina General del Burdwan Medical College, and Hospital, Burdwan, Bengala Occidental, India, entre mayo de 2018 y mayo de 2022. Resultados: Presentamos 11 pacientes (seis hombres y cinco mujeres) con mieloneuropatía de diferentes etiologías (deficiencias de vitamina B12, cobre y vitamina E, intoxicación por organofosforados, abuso crónico de alcohol, abuso de sustancias ilícitas, trastorno neurológico relacionado con anticuerpos anti-tiroglobulina / antiperoxidasa tiroidea que responde a esteroides, síndrome de Sjögren, infección por chikungunya, paraneoplásico y hereditario). Conclusión: El análisis meticuloso de la historia y del examen clínico, así como la utilización e interpretación adecuadas de los hallazgos bioquímicos, electrofisiológicos y de neuroimagen son condiciones sine qua non para un enfoque preciso y consistente para evaluar un caso sospechoso de mieloneuropatía, lo que facilita el tratamiento temprano y su recuperación. El diagnóstico diferencial de esta patología requiere un conocimiento del modo de inicio de los síntomas, su progresión, el patrón de hallazgos mielopáticos/neuropáticos y el reconocimiento de otras manifestaciones neurológicas o sistémicas. Las etiologías de las mieloneuropatías deben subdividirse en trastornos metabólicos (nutricionales), tóxicos (inducidos por toxinas), infecciosos, inflamatorios (mediados por mecanismos inmunitarios), paraneoplásicos y hereditarias.
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INTRODUCTION: Cadmium is an important heavy metal in neurobiology, with potential neurotoxic effects, often in the form of polyneuropathy (PNP). CASE REPORT: We present an exceptional case of PNP due to cadmium of toxic-occupational origin, specifically a 47-year-old man, aeronautical mechanic, with a 5-year clinical picture, consisting of a tingling sensation having a 'glove and stocking' distribution of symptoms and bimanual manipulative clumsiness. The neurological examination revealed bilateral achilles hyporeflexia and protopathic-thermal-algesic exteroceptive hypoesthesia in hands and feet. The following complementary rests were requested: toxic-metabolic-infectious-vitamin profile, full craniospinal MRI, electroneurographic-electromyographic study (ENG-EMG) of the upper and lower limbs, PET-CT body and 24-hour video-electroencephalogram. The results were consistent with an axonal, distal, symmetric sensory-motor PNP, of moderate intensity, chronic evolution, with active denervation, of toxic-occupational origin due to cadmium. The patient continued on sick leave to cease exposure to cadmium, initiating intensive multimodal neurorehabilitation program, with serial analytical determinations of toxins and new ENG-EMG studies every 6 months. With normalization of the altered values ??and complete clinical restitution at one-year follow-up. CONCLUSIONS: This case highlights the importance of including the toxicological determination of cadmium in case of suspicion of a PNP of toxic-occupational origin, once ruled out other etiologies, in order to early interrupt occupational exposure, as it is a potentially reversible cause of peripheral neuropathy. Currently there is no specific pharmacological treatment against cadmium tested in humans. Randomized clinical trials carried out in these patients are warranted to develop an anti-cadmium drug in refractory cases despite the end of exposure.
TITLE: Polineuropatía por cadmio: una causa infrecuente, pero no menos importante, de neuropatía periférica.Introducción. El cadmio es un metal pesado importante en neurobiología, con potenciales efectos neurotóxicos, frecuentemente en forma de polineuropatía. Caso clínico. Presentamos un caso excepcional de polineuropatía por cadmio de origen tóxico-ocupacional, en concreto, un varón de 47 años, mecánico aeronáutico, con un cuadro de cinco años de evolución, consistente en sensación de hormigueo 'en guante y calcetín' y torpeza manipulativa bimanual. En la exploración destacaba una hiporreflexia aquílea bilateral, y una hipoestesia exteroceptiva protopático-térmico-algésica en las manos y los pies. Se solicitó analítica general completa con perfil tóxico-metabólico-infeccioso-vitamínico, resonancia magnética craneomedular completa, estudio electroneurográfico-electromiográfico de los miembros superiores e inferiores, tomografía por emisión de positrones-tomografía axial computarizada body y videoelectroencefalograma de 24 horas. Los resultados fueron compatibles con una polineuropatía sensitivomotora axonal, distal, simétrica, de intensidad moderada, de evolución crónica y desnervación activa, de origen tóxico-ocupacional por cadmio. El paciente prosiguió la baja laboral para cesar la exposición al cadmio, iniciando neurorrehabilitación intensiva multimodal, y determinaciones analíticas seriadas de tóxicos y nuevos estudios electroneurográficos-electromiográficos cada seis meses, con normalización de los valores alterados y restitución clínica ad integrum al año. Conclusiones. Este caso enfatiza la importancia de incluir la determinación toxicológica del cadmio ante la sospecha de una polineuropatía de origen tóxico-ocupacional, descartadas otras etiologías, para interrumpir precozmente dicha exposición laboral, al ser una causa potencialmente reversible de neuropatía periférica. Actualmente no existe un tratamiento farmacológico específico frente al cadmio demostrado en seres humanos. Urgen ensayos clínicos aleatorizados en estos pacientes, para desarrollar un fármaco frente al cadmio en casos refractarios pese a finalizar la exposición.
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Exposição Ocupacional , Doenças do Sistema Nervoso Periférico , Polineuropatias , Cádmio/toxicidade , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Polineuropatias/induzido quimicamente , Polineuropatias/complicações , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/efeitos adversosRESUMO
INTRODUCTION: Very little has been written on seizure management in palliative care (PC). Given this situation, and considering the forthcoming setting up of the Palliative Care Unit at our neurorehabilitation centre, the Clínica San Vicente, we decided to establish a series of guidelines on the use of antiepileptic drugs (AEDs) for handling seizures in PC. METHODS: We conducted a literature search in PubMed to identify articles, recent manuals, and clinical practice guidelines on seizure management in PC published by the most relevant scientific societies. RESULTS: Clinical practice guidelines are essential to identify patients eligible for PC, manage seizures adequately, and avoid unnecessary distress to these patients and their families. Given the profile of these patients, we recommend choosing AEDs with a low interaction potential and which can be administered by the parenteral route, preferably intravenously. Diazepam and midazolam appear to be the most suitable AEDs during the acute phase whereas levetiracetam, valproic acid, and lacosamide are recommended for refractory cases and long-term treatment. CONCLUSIONS: These guidelines provide general recommendations that must be adapted to each particular clinical case. Nevertheless, we will require further well-designed randomised controlled clinical trials including large samples of patients eligible for PC to draft a consensus document recommending adequate, rational, and effective use of AEDs, based on a high level of evidence, in this highly complex area of medical care.
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Anticonvulsivantes/uso terapêutico , Carbamazepina/uso terapêutico , Guias como Assunto , Cuidados Paliativos/métodos , Convulsões/tratamento farmacológico , Humanos , Levetiracetam , Ácido Valproico/uso terapêuticoRESUMO
INTRODUCTION: Genitourinary cancers constitute a heterogeneous and increasingly frequent group of malignant tumors that have the potential to derive directly, or indirectly from the treatment applied, in a series of neurological complications that negatively impact on the quality of life of the patients who suffer them. AIMS: To report the most relevant data on the main neurological complications of genitourinary cancers. DEVELOPMENT: We conducted a PubMed search for articles, latest books, leading clinical practice guidelines, and scientific societies, regarding the appearance of such complications. CONCLUSIONS: Neurological complications of genitourinary cancers generate a significant burden of morbidity and mortality in cancer patients. In a paradoxical manner, owing to the raised survival of these patients, the likelihood of metastatization at the nervous system level and/or adverse effects related to the treatment received, especially due to chemotherapy, is also increased. Currently, diagnosis and management of neurological complications associated with genitourinary cancers represent a very important area of growing interest for the development of research projects that allow to improve the prognosis and quality of life genitourinary cancers subjects and their relatives and/or caregivers. For this purpose, it is necessary to know more about the etiopathogenesis and pathophysiology that leads to the occurrence of these type of complications in genitourinary cancers individuals, in particular paraneoplastic syndromes. Moreover, on the other hand, to carry out further well-designed randomized controlled clinical trials that expand the therapeutic arsenal with new chemotherapeutic drugs that possess a better antineoplastic effectiveness and improve the safety related to the neurotoxic side effects.
TITLE: Principales complicaciones neurologicas de los canceres nefrourologicos.Introduccion. Los canceres nefrourologicos constituyen un conjunto heterogeneo y cada vez mas frecuente de tumores malignos que poseen el potencial de derivar directamente, e indirectamente por el tratamiento aplicado, en una serie de complicaciones neurologicas que impactan negativamente sobre la calidad de vida de los pacientes. Objetivo. Exponer los datos mas relevantes sobre las principales complicaciones neurologicas de los canceres nefrourologicos. Desarrollo. Busqueda de articulos en PubMed, ultimos libros y principales guias de practica clinica y sociedades cientificas publicados referentes al diagnostico y tratamiento de dichas complicaciones. Conclusiones. Las complicaciones neurologicas de los canceres nefrourologicos generan una carga importante de morbimortalidad en los pacientes oncologicos. Paradojicamente, gracias al aumento de su supervivencia, tambien se incrementa la probabilidad de producirse metastasis en el sistema nervioso o efectos adversos por el tratamiento, en especial la quimioterapia. Actualmente, el diagnostico y el tratamiento de las complicaciones neurologicas asociadas a los canceres nefrourologicos suponen un area muy importante de interes creciente para el desarrollo de trabajos de investigacion que permitan mejorar el pronostico y la calidad de vida de estos pacientes y de sus familiares o cuidadores. Para ello, es preciso conocer mejor la etiopatogenia y la fisiopatologia que llevan a la aparicion de este tipo de complicaciones, particularmente los sindromes paraneoplasicos, y, por otro lado, la realizacion de ensayos clinicos controlados, aleatorizados, bien diseñados, que amplien el arsenal terapeutico con nuevos farmacos quimioterapicos con mayor efectividad antineoplasica y mejor seguridad relativa a los efectos secundarios neurotoxicos.
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Neoplasias Renais/complicações , Doenças do Sistema Nervoso/etiologia , Neoplasias da Próstata/complicações , Neoplasias Testiculares/complicações , Neoplasias Encefálicas/secundário , Humanos , Neoplasias Renais/patologia , Masculino , Neoplasias da Próstata/patologia , Neoplasias da Coluna Vertebral/secundário , Neoplasias Testiculares/patologiaRESUMO
BACKGROUND: Recent postmortem studies have demonstrated pathological changes, including Purkinje cell loss, in the cerebellum in essential tremor (ET). Toxic exposures that compromise cerebellar tissue could lower the threshold for developing ET. Ethanol is a well-established cerebellar toxin, resulting in Purkinje cell loss. OBJECTIVE: To test whether higher baseline ethanol consumption is a risk factor for the subsequent development of incident ET. METHODS: Lifetime ethanol consumption was assessed at baseline (1994-1995) in a prospective, population-based study in central Spain of 3285 elderly participants, 76 of whom developed incident ET by follow-up (1997-1998). RESULTS: In a Cox proportional hazards model adjusting for cigarette pack-years, depressive symptoms and community, the baseline number of drink-years was marginally associated with a higher risk of incident ET (relative risk, RR = 1.003, p = 0.059). In an adjusted Cox model, the highest baseline drink-year quartile doubled the risk of incident ET (RR = 2.29, p = 0.018), while other quartiles were associated with more modest elevations in risk (RR(3rd quartile) = 1.82 (p = 0.10), RR(2nd quartile) = 1.75 (p = 0.10), RR(1st quartile) = 1.43 (p = 0.34) vs non-drinkers (RR = 1.00)). With each higher drink-year quartile, the risk of incident ET increased an average of 23% (p = 0.01, test for trend). CONCLUSIONS: Higher levels of chronic ethanol consumption increased the risk of developing ET. Ethanol is often used for symptomatic relief; studies should explore whether higher consumption levels are a continued source of underlying cerebellar neurotoxicity in patients who already manifest this disease.
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Consumo de Bebidas Alcoólicas/epidemiologia , Tremor Essencial/epidemiologia , Idoso , Depressão/complicações , Depressão/epidemiologia , Feminino , Humanos , Estilo de Vida , Masculino , População , Modelos de Riscos Proporcionais , Estudos Prospectivos , Medição de Risco , Fatores de Risco , Fumar/epidemiologia , Fatores Socioeconômicos , Espanha/epidemiologia , Inquéritos e QuestionáriosRESUMO
Glutathione-S-transferases (GST) are polymorphic enzymes that participate in the metabolism of carcinogens (including those of tobacco smoke) and pesticides. We investigated the possible association between the GSTP1 genotype and allelic variants and the risk for essential tremor (ET). We studied the frequency of the GSTP1 genotypes and allelic variants in 200 patients with ET and 220 healthy controls using PCR-RFLP method. The association between GSTP1 polymorphism and the exposure to some environmental factors (agricultural work, pesticides, well-water and smoking-cigarettes habit) was also studied in a subgroup of patients. The frequencies of the GSTP1 genotypes and allelic variants did not differ significantly between patients with ET and controls or between patients with ET exposed to agricultural work, well water and cigarette smoking versus those non-exposed. Mutated allelic variants were significantly more frequent in patients with ET exposed to pesticides versus those non-exposed. GSTP1 polymorphism was unrelated with the age of onset of ET. GSTP1 genotypes and allelic variants were not related with the risk for ET with the possible exception of those patients exposed to pesticides.
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Tremor Essencial/etiologia , Tremor Essencial/genética , Predisposição Genética para Doença , Glutationa S-Transferase pi/genética , Polimorfismo Genético/genética , Risco , Adulto , Idoso , Feminino , Frequência do Gene , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Praguicidas/toxicidadeAssuntos
Carcinoma de Células Pequenas/complicações , Síndrome de Guillain-Barré/etiologia , Neoplasias Pulmonares/complicações , Idoso , Carcinoma de Células Pequenas/diagnóstico , Diagnóstico Diferencial , Feminino , Síndrome de Guillain-Barré/diagnóstico , Humanos , Neoplasias Pulmonares/diagnósticoRESUMO
for a long time to the research field despite its unquestionable diagnostic value. The availavility of programs able to automatically obtain a spectrum, and the current possibility of estimating easily the relationship among its different peaks, have approached this diagnostic technology to the current clinical situation. With hydrogen MRI spectroscopy (MRS) it is possible to obtain additional information which make it possible to distinguish among different neurological alterations with similar morphological appearance, such as cerebral tumors and pseudotumoral types of inflammatory processes, or tumor recurrence and radionecrosis. On other occasions, it makes it possible to detect alterations which are invisible by imaging study, such as medial temporal sclerosis, or multiple sclerosis. It is also very useful in following-up many alterations, either in their natural history, as in Alzheimers disease, or in order to monitor treatments on cerebral tumors or infectious processes. However, MRS does not often show pathognomonic patterns, so it is always recommended to consider it not just in the clinical context, but as inseparable part of an MR study, either structural or functional (diffusion, perfusion), since it is this is how it provides more useful information from a clinical point of view.
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Imageamento por Ressonância Magnética/métodos , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/patologia , Neurologia/instrumentação , Humanos , HidrogênioRESUMO
Magnetic resonance imaging (MRI) has allowed us to establish a set of radiologic signs associated with intracranial hypotension syndrome. Findings are partly influenced by cerebral displacement. Intracranial hypotension syndrome is characterized by a decrease in cerebrospinal fluid (CSF) pressure to less than 60 mm H2O associated with occipital headache radiating to the frontal and temporal zones. For diagnostic purposes, the most common cause is anesthetic or therapeutic dural puncture, although spontaneous CSF leakage can occur. CSF protein and lymphocyte counts may be high, while the cranial meninges biopsy is normal. MRI images may show a descended brain, taking the start of the sylvian aqueduct and the location of the cerebellar amygdalae as points of reference; diminished size of the subarachnoidal cisterns and occasionally of the cerebral ventricles; meningeal enhancement from increased uptake of the contrast solution; subdural hygromas and hematomas; and pituitary enlargement. Paraspinal fluid and dilated epidural veins may be observed. Radiologic images and clinical signs are related. When CSF pressure is very low, there is greater meningeal enhancement, subdural collection and cerebral displacement. Findings gradually disappear as symptoms diminish. The signs and symptoms that might develop during intracranial hypotension syndrome vary according to the brain structure that might be affected during descent, repositioning and the traction of anchoring structures. MRI allows the degree of cerebral and spinal involvement to be ascertained, to predict whether resolution of the clinical picture will be early or late and to visualize the effect of approaches to reducing CSF leakage.
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Dura-Máter/lesões , Cefaleia/etiologia , Hipotensão Intracraniana/etiologia , Imageamento por Ressonância Magnética , Punções/efeitos adversos , Derrame Subdural/etiologia , Raquianestesia/efeitos adversos , Ventrículos Cerebrais/patologia , Diagnóstico Diferencial , Encefalocele/etiologia , Encefalocele/patologia , Cefaleia/patologia , Hematoma Subdural Agudo/etiologia , Hematoma Subdural Agudo/patologia , Humanos , Hipotensão Intracraniana/diagnóstico , Hipotensão Intracraniana/patologia , Imageamento por Ressonância Magnética/métodos , Meninges/patologia , Mielografia/efeitos adversos , Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Punção Espinal/efeitos adversos , Derrame Subdural/diagnóstico , Derrame Subdural/patologiaAssuntos
Dor Lombar/complicações , Atrofia Muscular/etiologia , Adulto , Biópsia , Doença Crônica , Humanos , Dor Lombar/reabilitação , Imageamento por Ressonância Magnética , Masculino , Proteínas Musculares/análise , Músculo Esquelético/química , Músculo Esquelético/patologia , Atrofia Muscular/diagnóstico , Atrofia Muscular/diagnóstico por imagem , Atrofia Muscular/patologia , Tomografia Computadorizada por Raios XRESUMO
We report a case of cluster headache in a patient with a macroprolactinoma. Symptomatic cluster headache was suspected because of an unsatisfactory response to medications that are usually effective in idiopathic cluster headache. The neurological examination was normal. However, magnetic resonance imaging demonstrated a large pituitary tumor. One year after starting treatment with cabergoline, the patient remains asymptomatic. Symptomatic cluster headache should be suspected when the clinical features of the headache are atypical. By inducing vascular mechanisms, the parasellar lesion may have played a role in initiating the cluster headache.
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Cefaleia Histamínica/etiologia , Neoplasias Hipofisárias/complicações , Prolactinoma/complicações , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Hipofisárias/diagnóstico , Prolactinoma/diagnósticoRESUMO
INTRODUCTION: The diagnosis of a cerebral abscess is a real challenge since the clinical and radiological findings are often non-specific and undistinguishable from those seen with cystic or necrotic tumours. Recently it has been suggested that diffusion sequences may be useful in the differential diagnosis of a necrotic or cystic mass. PATIENTS AND METHODS: Nine patients with cystic or necrotic intracranial masses were studied. The diagnoses were: three pyogenic abscesses, three metastases and three high grade gliomas. The diffusion images were evaluated visually and by means of maps based on the apparent diffusion coefficient (CDA). RESULTS: All lesions showed fine iso-intense or slightly hyperintense walls in T1 potentiated sequences, and isointense or slightly hypointense walls in T2 potentiated sequences. In all cases the wall took up gadolinium intensity, with a well-defined smooth edge (ring uptake). In the diffusion sequences the abscesses showed a very strong central signal, as compared with the low signal of other lesions. The CDA were significantly lower in the abscesses than in the tumours. CONCLUSIONS: Since cerebral abscesses are potentially curable, early diagnosis should be made. A cerebral abscess should be suspected in all cases of cystic or necrotic masses with hypersignals in diffusion sequences and low CDA.
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Abscesso Encefálico/patologia , Neoplasias Encefálicas/patologia , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , NecroseAssuntos
Acetatos/uso terapêutico , Aminas , Analgésicos/uso terapêutico , Ácidos Cicloexanocarboxílicos , Neuropatias Diabéticas/tratamento farmacológico , Cuidados Paliativos , Ácido gama-Aminobutírico , Doença Aguda , Idoso , Neuropatias Diabéticas/fisiopatologia , Feminino , Gabapentina , Humanos , Dor/fisiopatologiaRESUMO
INTRODUCTION: Lhermitte's sign was first described by Pierre Marie and Chatelin in 1917. Lhermitte published his report in 1920 and reviewed this in 1924. This phenomenon is characterized by the occurrence of an electric shock-like sensation going along the spine in a cervico-caudal direction with flexion of the neck, and may also be felt in the upper and lower limbs. Clinical cases. Case 1. A 49 year-old woman diagnosed as having breast cancer and being treated with cisplatin presented with Lhermitte's sign. On physical examination the osteotendinous reflexes were absent but the abdominal cutaneous reflexes were present. There was reduced sensitivity to vibration. Case 2. An 80 year-old man, previously operated on for adenocarcinoma of the colon, without further treatment, presented with progressive weakness of all four limbs and Lhermitte's syndrome. On examination there was obvious weakness of all four limbs, with the sensory level at C3. A cervical MR scan showed a metastasis in the vertebral body of C3 with epidural involvement and compression of the spinal cord. Case 3. A 54 year-old man was being treated by radiotherapy for cancer of the larynx. He presented at the onset of Lhermitte's sign but MR and physical examination were normal. CONCLUSION: Lhermitte's sign is non-specific, although in oncological patients a detailed clinical history and examination should be done seeking data regarding chemotherapy, radiotherapy and spinal compression.