Vasculitis reumatoidea como manifestación extraarticular en dos casos / Rheumatoid vasculitis as an extra articular manifestation in two cases

La vasculitis reumatoidea es un proceso inflamatorio poco frecuente que confiere una morbilidad y mortalidad significativa en pacientes con artritis reumatoidea (AR). Presenta una incidencia de 0,7 a 5,4% de los casos de AR y hasta un 40% de mortalidad a 5 años, siendo la manifestación extraarticular de mayor gravedad1,2. Se caracteriza por el desarrollo de vasculitis necrotizante; ocurre típicamente en pacientes masculinos con AR seropositiva de larga data, nodular y erosiva, tabaquistas y, en general, tiene mal pronóstico. Se presenta una serie de casos, paciente masculino y femenino con AR y manifestaciones extraarticulares, necrosis digital y compromiso multiorgánico, interpretados como cuadros de vasculitis reumatoidea, realizando tratamiento de referencia con inmunosupresores.

Rheumatoid vasculitis is a rare inflammatory process that confers significant morbidity and mortality in patients with rheumatoid arthritis (RA); it has an incidence of 0.7 to 5.4% of RA cases and up to 40% mortality at five years, making it the most serious of all the extra-articular manifestations of RA1,2. It is characterized by the development of necrotizing vasculitis, it typically occurs in male patients with long-standing seropositive RA, erosive nodular, smokers and generally has a poor prognosis. A series of cases is presented, male and female with RA and extra-articular manifestations, digital necrosis and multiorgan system involvement, interpreted as rheumatoid vasculitis, undergoing treatment with immunosuppressants.

Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell's phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.

Constrictive entrapment neuropathies of a limb secondary to restraint strapping: A case report

@#<p style="text-align: justify;"><strong>Rationale:</strong> Entrapment neuropathies are peripheral nerve disorders at specific anatomical locations. They may be caused by trauma in a manner of sprains or bone fracture, but it is often caused by repetitive insults or compression of nerves as they travel through a narrow anatomic space. Pregnancy and pre-existing comorbidities such as diabetes, obesity, cancer, or autoimmune diseases may also cause nerve entrapment.</p><p style="text-align: justify;"><strong>Objective:</strong> To highlight the case of a 52-yearold female developing right foot dysesthesia and weakness after continuous restraint strapping from her previous hospitalization.</p><p style="text-align: justify;"><strong>Case:</strong> Here we have the case of a 52-year-old Filipino female consulted because of right foot dysesthesia, allodynia, and mild weakness. She had a history of bipolar disorder and recent onset of acute psychosis and overdosing with her irregularly taken maintenance olanzapine tablets. She was put on restraint strapping of the right lower limb in her one-week hospital stay. This resulted in developing restraint marks on her right ankle accompanied by difficulty walking on heels and toes, spontaneous dysesthesia, and touch allodynia of her entire right foot. An electrodiagnosis yielded right lower limb focal neuropathies involving the right fibular nerve, right tibial nerve, right superficial fibular, and right sural nerves. The prescribed amitriptyline and gabapentin for 6 months led to gradual improvement of neuropathic pain.</p><p style="text-align: justify;"><strong>Discussion and Summary:</strong> Our case exemplifies focal limb neuropathies from entrapment due to restraint strapping. Electrodiagnostic confirmation of neuropathies of the same limb sensory and motor nerves was mandated to corroborate clinical neuropathic pain and after ruling out other causes of entrapment neuropathies. Prolonged use of neuropathic pain medications were needed to attain relief in this present case. Restrictive strapping is an iatrogenic cause of entrapment neuropathy that is preventable, had there been proper medical attention applied.</p>

Polineuropatia desmielinizante como manifestação inicial isolada de hanseníase: relato de caso / Demyelinating polyneuropathy as an isolated initial manifestation of leprosy: case report

RESUMO: Modelo do Estudo: Relato de caso. Importância do problema: No mundo, mais de três milhões de pessoas estão vivendo com deficiência física devido à hanseníase. O Brasil é o segundo país com o maior número de casos novos registrados.A magnitude e o alto risco de incapacidade mantêm a doença como problema de saúde pública. O diagnóstico de hanseníase em geral é simples. Porém, quadros com ausência de lesões cutâneas características, somente com alterações neurais, representam um desafio para o diagnóstico diferencial com outras doenças neurológicas. Comentários: Relatamos o caso de um paciente encaminhado ao serviço de neurologia com história clínica e eletroneuromiografia compatíveis com polineuropatia desmielinizante, sem qualquer lesão cutânea ao exame de admissão. O raciocínio clínico inicial foi direcionado para o diagnóstico das polineuropatias desmielinizantes inflamatórias adquiridas como Polineuropatia Desmielinizante Inflamatória Crônica (CIDP) e suas variantes. No entanto, após anamnese e exame físico detalhados, chamou a atenção a ausência do componente atáxico e a presença predominante de alterações sensitivas de fibra fina, espessamento de nervo e importante fator epidemiológico para hanseníase, motivando a suspeita e a in-vestigação desta enfermidade por meio da biópsia de nervo que foi sugestiva de hanseníase. Após três meses, em novo exame do paciente para biopsiar áreas de anestesia para reforçar o diagnóstico, observou-se o surgimento de extensas lesões levemente hipocrômicas no tronco e membros inferiores, cuja biópsia definiu o diagnóstico de hanseníase. (AU)

ABSTRACT: Study: Case report. Importance: Worldwide over three million people are living with disabilities due to leprosy. Brazil is the second country with the highest number of new cases registered. The magnitude and high risk of disability make the disease a public health problem. The diagnosis of leprosy can be simple. However, in the absence of skin lesions and with many possibilities of neurological impairment, diagnosis can become a challenge. Comments: We report the case of a patient referred to the neurology service with a clinical history and electrophysiological tests compatible with demyelinating polyneuropathy, without any skin lesion at admission examination. The initial clinical research was directed to the diagnosis of acquired inflammatory demyelinating polyneuropathies such as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)and its variants. However, after anamnesis and detailed physical examination, the absence of the ataxic component and the predominant presence of sensory alterations of fine fiber, nerve thickening and important epidemiological factor for leprosy,led to the suspicion and investigation of this disease by nerve biopsy that was suggestive of leprosy. After three months, in a new patient examination "to perform a biopsy in areas of anesthesia" to reinforce the diagnosis, there was the appearance of extensive slightly hypochromic lesions in the trunk and lower limbs, whose biopsy defined the diagnosis of leprosy.(AU)

Treatment Strategies for Diabetic Neuropathy / 대한신경근육질환학회지

The most prevalent microvascular complication of diabetes mellitus is neuropathy, which encompasses distal symmetric polyneuropathy, mononeuropathy, radiculoplexopathy, and autonomic neuropathy. Intensive glucose control prevents and effectively halts the progression of diabetic neuropathy in patients with type 1 diabetes mellitus. However, the effect of strict glucose control itself is at modest in those with type 2 diabetes. Although we have better understanding of the mechanism of diabetic neuropathy, many pharmacologic trials for the targeting underlying nerve damage have reported unsuccessful results. In this review, the effects and limitations of the current therapeutic options will be discussed.

Sensory multiple mononeuropathy induced by pravastatin use: from a case report to literature's review / Mononeuropatia múltipla sensitiva induzida pelo uso de pravastatina: de um relato de caso à revisão de literatura

Statins are frequently prescribed in clinical practice for their proven efficacy in prevention of cardiovascular and cerebrovascular diseases. Despite the recognized beneficial effects of this class of drugs, in recent years, many studies published in medical literature have shown a wide range of adverse effects as a consequence of this therapy, including the risk of peripheral neuropathy. The purpose of this article is to report a case in which clinical features consistent with multiple mononeuropathy probably secondary to use of pravastatin were observed. The case report is followed by a review of the relevant literature.

As estatinas são frequentemente prescritas na prática clínica por sua comprovada eficácia na prevenção de doenças cardiovasculares e cérebrovasculares. Apesar dos reconhecidos efeitos benéficos dessa classe medicamentosa, nos últimos anos, diversos estudos publicados na literatura médica vem evidenciando uma ampla variedade de efeitos colaterais como consequência desta terapia, incluindo o risco de neuropatias periféricas. O objetivo deste artigo é relatar um caso no qual foram observadas manifestações clínicas compatíveis com o diagnóstico de mononeuropatia múltipla sensitiva, provavelmente secundária ao uso de pravastatina. O relato de caso é acompanhando de uma revisão de dados pertinentes da literatura.

Henoch-Schönlein Purpura Presenting as Mononeuritis Multiplex

No abstract available.

Successful additional clarithromycin and tacrolimus treatment for hypereosinophilia associated with eosinophilic granulomatosis with polyangiitis

A 41-year-old man suffering from eosinophilic granulomatosis with polyangiitis (EPGA), diagnosed at another clinic on the basis of American College of Rheumatology Criteria, with a history of bronchial asthma, eosinophilia, mononeuritis multiplex, and non-fixed pulmonary infiltrates, was admitted to our department for further treatment. The patient complained of chest pain that started recently. An echocardiogram identified myocardial thickening and decreased wall motion, based on which the patient was diagnosed as having EPGA with myocarditis. The patient was successfully treated using glucocorticoids, such as methyl prednisolone (PSL) and PSL in combination with cyclophosphamide (CPM). However, CPM administration was discontinued afterwards because of the risk of bone marrow toxicity, the increased eosinophilic count (EOC) that we considered as an index of disease activity. Subsequently, the patient received additional clarithromycin (CAM) and tacrolimus (TAC) treatment considering their immunomodulatory effects. As a result, the EOC decreased and the PSL dosage could be reduced. This case shows that additional CAM and TAC treatment may be beneficial in some cases of EPGA.

Extended duration pulsed radiofrequency for the management of refractory meralgia paresthetica: a series of five cases

Meralgia paresthetica (MP) is a sensory mononeuropathy, caused by compression of the lateral femoral cutaneous nerve (LFCN) of thigh. Patients refractory to conservative management are treated with various interventional procedures. We report the first use of extended duration (8 minutes) pulsed radiofrequency of the LFCN in a case series of five patients with refractory MP. Four patients had follow up for 1–2 years, and one had 6 months follow up. All patients reported remarkable and long lasting symptom relief and an increase in daily life activities. Three patients came off medications and two patients required minimal doses of neuropathic medications. No complications were observed.

An Unusual Case of Bilateral Meralgia Paresthetica Following Femoral Cannulations / 신경중재치료의학

Meralgia paresthetica (MP) is a sensory mononeuropathy of the lateral femoral cutaneous nerve (LFCN). MP has rarely been reported after a femoral intervention approach. We report a case of bilateral meralgia paresthetica following bilateral femoral cannulation. A 64-year-old male received cardiac catheterization and treatment via a bilateral femoral vein. After cardiac catheterization, the patient presented with paresthesia in the anterolateral aspect of the bilateral thigh. After performing nerve conduction studies and electromyography, he was diagnosed as MP. Although a bilateral LFCN lesion following a femoral approach is very rare, MP might require caution regarding potential variations in LFCN when performing the femoral approach.

Vasculitis livedoide asociada a mononeuritis múltiple / Livedoid vasculopathy associated with mononeuritis multiplex

Se presenta un varón de 64 años, obeso, hipertenso, con diabetes no-insulinodependiente de 6 años de evolución. Se presentó a la consulta con úlceras dolorosas en miembros inferiores de aparición estival, de 10 meses de evolución y relataba parestesias de 20 meses de evolución. La electromiografía reveló la presencia de una mononeuritis múltiple que luego progresó a polineuropatía axonalmixta. Necrosis dérmica asociada a trombos fibrinoides, depósitos de material hialino con infiltrado inflamatorio mixto fue observado en la biopsia de piel, llegándose al diagnóstico de vasculitis livedoide. La misma no sería una verdadera vasculitis, sino un fenómeno vaso-oclusivo que debe diferenciarse de otras vasculitis (vasculitis cutáneas de pequeños vasos, panarteritis nodosa, síndrome antifosfolipídico, estasis venoso crónico, pioderma gangrenoso, entre otros). El compromiso neurológico es excepcional en esta vasculopatía, habiendo sido descriptos sólo 5 casos con esta asociación

Concurrent meralgia paresthetica and radiculopathy of the left leg: A case report

Meralgia paresthetica (MP) is a painful mononeuropathy of the lateral femoral cutaneouse nerve (LFCN) characterized by localized symptoms of numbness, tingling, pain and paresthesia along the anterolateral thigh area. L4 and L5 radiculopathy is set of symptoms that include sharp, burning or shooting pain, which is usually localized to anterolateral leg area and along the dermatomal distribution. When symptoms of MP and lumbar disc disease occur together it is not easy to diagnose MP. We report a case of synchronous post-traumatic MP and radiculopathy due to intervertebral disc herniation at L3–4 and 4–5. A 59-year-old male patient was admitted to the emergency room with symptoms of low back pain with left severe L4, L5 radiculopathy. This patient also complained of numbness and paresthesia in the left anterolateral thigh. After detailed history taking and lateral femoral cutaneouse nerve block, he was diagnosed with MP.

Common Peroneal Neuropathy With Anterior Tibial Artery Occlusion: A Case Report

Peroneal neuropathy is a common mononeuropathy of the lower limb. Some studies have reported cases of peroneal neuropathy after vascular surgery or intervention. However, no cases of peroneal neuropathy with occlusion of a single peripheral artery have been previously reported. A 73-year-old man was referred with a 3-week history of left-sided foot drop. He had a history of valvular heart disease and arrhythmia, and had previously been treated with percutaneous coronary intervention. Computed tomography angiogram of the lower extremity showed proximal occlusion of the left anterior tibial artery. An electrodiagnostic study confirmed left common peroneal neuropathy. After diagnosis, anticoagulation therapy was started and he received physical therapy.

Utilidad de la neurografía por IRM en el diagnóstico de la meralgia parestésica / Usefulness of MRI neurography in the diagnosis of meralgia paresthetica

Objetivo: Evidenciar cómo la Imagen Resonancia magnética (IRM) con equipo de 3 Tesla, utilizando secuencias neurográficas específicas y uso de contraste endovenoso permite ubicar el área de lesión nerviosa. Caso clínico: Mujer de 17 años con hipoestesia y parestesias en la región anterior y lateral del muslo izquierdo, se le realiza un estudio de Resonancia Magnética (RM) en equipo Philips Achieva 3 T, usando protocolo neurográfico y contraste endovenoso, demostrando engrosamiento y cambios de señal del nervio y realce post-contraste característico de la neuropraxia. Conclusión: el caso presentado demuestra el valor actual de los estudios de IRM con técnica neurográfica en la evaluación de la patología de los nervios periféricos. Es fundamental utilizar secuencias adecuadas, realizar un estudio comparativo bilateral y en lo posible usar medios de contraste endovenoso para aumentar la sensibilidad. Estos hallazgos resultan de gran importancia a la hora de planificar la estrategia quirúrgica.

Objective: Demonstrate the usefulness of Magnetic Resonance Imaging (MRI) with equipment 3 Tesla using neurographics specific sequences and use of intravenous contrast can locate the area of nerve injury.Clinical case: 17 year old woman with hypoesthesia and paresthesia in the anterior and lateral region of the left thigh, the study of Magnetic Resonance Imaging (MRI) equipment Philips Achieva 3 T, using neurographic protocol and intravenous contrast, showing thickening and changes signal nerve and post-contrast enhancement characteristic in neuropraxia.Conclusión: the case presented shows the current value of MRI studies with neurographics technique in assessing the pathology of peripheral nerves. It is essential to use appropriate sequences, make a bilateral comparative study and possible use of intravenous contrast media to increase sensitivity. These findings are of great importance when planning the surgical strategy.

Mono-neuropathie multiple motrice aiguë révélant une neurosyphilis chez une patiente VIH négative A propos d'un cas

La mono-neuropathie multiple est une atteinte successive et simultanée de plusieurs troncs nerveux, et la syphilis est une Infection Sexuellement Transmissible (IST), qui a vu sa prévalence décroître significativement avec la penicillinothérapie intraveineuse. La neuro-syphilis est l'une des formes les plus sévères de cette pathologie, caractérisée par un polymorphisme clinique et par des troubles neurologiques chroniques non-spécifiques. Nous rapportons une manifestation rare à type mono-neuropathie multiplemotrice aiguë. Il s'agit d'une patiente de 19 ans hospitalisée dans le service de Neurologie du Centre Hospitalier Universitaire de Fann, pour paraplégie flasque d'installation rapidement progressive,sans antécédents particuliers dans le post-partum immédiat. La sérologie syphilitique notamment(TPHA, VDRL) était fortement positive dans le sang et le LCR. L'EMG avait mis en évidence une sévère neuropathie axonale motrice des membres inférieurs, asymétrique avec importante dénervation et des paramètres sensitifs normaux. L'évolution était favorable sous pénicilline G. La neurosyphilis reste une pathologie à laquelle il faut penser dans notre contexte, elle peut revêtir des aspects trompeur

Essential Cryoglobulinemia Presenting as Polyneuropathy and Cerebral Infarction

Cryoglobulinemia is a rare medical condition defined by presence of cryoglobulins in serum and it may cause hyperviscosity syndrome or systemic vasculitis in variable organs including the skin, joints, liver, kidneys, lungs and nervous system. A vasculitic polyneuropathy or mononeuritis multiplex commonly occurs. However, central nervous system complications of cryoglobulinemia including stroke, encephalopathy and seizures are rarely reported. We represent a case of essential cryoglobulinemic vasculitis manifested as a sensorimotor polyneuropathy combined with an acute ischemic stroke attack.

The Diagnostic Dilemma of Neurolymphomatosis

Neurolymphomatosis (NL) defined as infiltration of the central nervous system or the peripheral nervous system (PNS) by malignant lymphoma cells is a rare clinical entity. However, the increasing use of fluorodeoxyglucose positron-emission tomography (FDG-PET) and magnetic resonance imaging in evaluating PNS disorders is resulting in; this condition being recognized more frequently. Here; we report five NL patients and review the current literature. We report five patients with non-Hodgkin's lymphoma (NHL) and NL, all of whom were men aged 47-69 years. The clinical presentation varied from symmetrical peripheral neuropathy to mononeuropathy. Peripheral neuropathy was the presenting manifestation of a systemic lymphoma in two patients (40%). Neuroimaging as well as whole-body FDG-PET helped in determining the correct diagnosis in all of the patients. NL is an unusual presentation of NHL resulting from infiltration of the PNS by malignant lymphomatous cells. While evaluating peripheral neuropathy, a high degree of suspicion of NL is required since the presenting symptoms vary, conventional radiology has only modest sensitivity, and a pathological diagnosis is often difficult. FDG-PET helps in the early diagnosis and treatment of this condition.

The Diagnostic Dilemma of Neurolymphomatosis

Neurolymphomatosis (NL) defined as infiltration of the central nervous system or the peripheral nervous system (PNS) by malignant lymphoma cells is a rare clinical entity. However, the increasing use of fluorodeoxyglucose positron-emission tomography (FDG-PET) and magnetic resonance imaging in evaluating PNS disorders is resulting in; this condition being recognized more frequently. Here; we report five NL patients and review the current literature. We report five patients with non-Hodgkin's lymphoma (NHL) and NL, all of whom were men aged 47-69 years. The clinical presentation varied from symmetrical peripheral neuropathy to mononeuropathy. Peripheral neuropathy was the presenting manifestation of a systemic lymphoma in two patients (40%). Neuroimaging as well as whole-body FDG-PET helped in determining the correct diagnosis in all of the patients. NL is an unusual presentation of NHL resulting from infiltration of the PNS by malignant lymphomatous cells. While evaluating peripheral neuropathy, a high degree of suspicion of NL is required since the presenting symptoms vary, conventional radiology has only modest sensitivity, and a pathological diagnosis is often difficult. FDG-PET helps in the early diagnosis and treatment of this condition.

Progressive Bilateral Facial Palsy as a Manifestation of Granulomatosis With Polyangiitis: A Case Report

Bilateral facial palsy, which is usually combined with other diseases, occurs infrequently. It may imply a life-threatening condition. Therefore, the differential diagnosis of bilateral facial palsy is important. However, the etiology is variable, which makes diagnosis challenging. We report a rare case of progressive bilateral facial palsy as a manifestation of granulomatosis with polyangiitis (GPA). A 40-year-old male with otitis media and right facial palsy was referred for electroneurography (ENoG), which showed a 7.7% ENoG. Left facial palsy occurred after 2 weeks, and multiple cavitary opacities were noted on chest images. GPA was diagnosed by lung biopsy. His symptoms deteriorated and mononeuropathy multiplex developed. The possibility of systemic disease, such as GPA, should be considered in patients presenting with bilateral facial palsy, the differential diagnosis of which is summarized in this report.