Case report of acupuncture and medication for a patient with bilateral peripheral facial paralysis as suspected case of neurosarcoidosis / 全日本鍼灸学会雑誌

[Objective] This report describes a patient with bilateral peripheral facial paralysis who was strongly suspected to have neurosarcoidosis and whose facial paralysis improved after acupuncture treatment.[Case] A 73-year-old man developed right facial nerve paralysis on July 24, X followed by left facial nerve paralysis three days later. The patient visited our Department of Neurology on August 2 and was hospitalized the next day. Upon examination, neurosarcoidosis was strongly suspected, and steroid pulse therapy was performed until he was discharged from the hospital on August 26. On September 13, still experiencing bilateral facial paralysis, the patient was referred to our department for acupuncture treatment. The patient's height was 159 cm and weight was 48.6 kg. Valley signs and pathologic reflexes were negative, and deep tendon reflexes and sensory tests of the upper and lower extremities were normal. MMT was fair only for the dorsiflexion movement of the left ankle. The Yanagihara score was 26 on the right and 10 on the left. Facial muscle contraction response to electrical acupuncture stimulation targeting the facial nerve showed contraction at 0.04 mA on the right side and no contraction at 0.30 mA on the left side. According to these examinations, the right side was considered to be mildly paralyzed and the left side was considered to be severely paralyzed, and acupuncture was performed to improve the symptoms of paralysis and prevent sequelae.[Treatment] Acupuncture was applied to GB2 and ST7 on the right side of the face. On the left side, acupuncture needles were placed on the frontalis muscle, orbicularis oculi muscle, elevator muscle of upper lip and wing of nose, nasalis muscle, zygomaticus major and minor muscle, orbicularis oris muscle, depressor angle oris muscle, and platysma muscle. From the 106th day of illness, asynchronous electroacupuncture was applied to the left side only. Acupuncture was performed once or twice a week for 10 minutes per session. The degree of paralysis was assessed by the Yanagihara score.[Progress] Acupuncture was combined with corticosteroids prescribed by the Department of Neurology. As a result, the right side had a score of 38 points after 170 days after start of illness and the left side had a score of 38 points after 204 days.[Discussion and Conclusion] The combined treatment of medication and acupuncture was effective and showed excellent results for this patient taking into account the degree of facial nerve damage. Acupuncture may be considered as an effective treatment option for bilateral peripheral facial paralysis.

A Rare Case of Disseminated Sarcoidosis Presenting with Neurosarcoidosis: A Case Report

@#Sarcoidosis is characterized by formation of inflammatory granulomas affecting all over the body, with pulmonary predilection (1). Neurosarcoidosis is a rare but potentially dangerous manifestation of sarcoidosis. We report a case of disseminated sarcoidosis presenting with a neurological diagnostic dilemma. Worsening mediastinal lymphadenopathy, together formation of lung and liver nodules making a sarcoidosis diagnosis favourable. Histology from these lesions showed non-caseating granulomatous inflammation. She was treated as a rare case of disseminated sarcoidosis. To date, there is no specific or clear guideline on the management of disseminated sarcoidosis.

Utilidad de la tomografia por emisión de positrones - tomografia computada para el diagnóstico de neurosarcoidosis. A propósito de un caso / Positron emission tomography/CT scanning in the diagnosis of neurosarcoidosis

Resumen El diagnóstico de la neurosarcoidosis es difícil por su polimorfismo clínico y radiológico, y cualquier parte del sistema nervioso central puede estar afectado. Presentamos el caso de una paciente joven con cefalea, meningitis linfocitaria subaguda, en quien a pesar de estudios bioquímicos e imagenológicos no se identificó la etiología. La tomografia por emisión de positrones con 18 fluorodeoxiglucosa permitió la identificación de un ganglio mediastinal hipermetabólico, con informe de patología de granulomas no caseificantes, hallazgos sugestivos de sarcoidosis, reflejando la utilidad de la tomografía por emisión de positrones con 18 fluorodeoxiglucosa para el diagnóstico y estadificación de la enfermedad, especialmente en las situaciones en las que no hay evidencia mediante otros estudios imagenológicos de enfermedad extraneural.

Abstract The diagnosis of neurosarcoidosis is difficult because of its clinical and radiological polymorphism. Any part of the central nervous system may be affected, and imaging studies are usually inconclusive. The case is presented of a young patient with headache, bilateral facial paralysis, and subacute lymphocytic meningitis in whom, despite biochemical and imaging studies the origin was not identified. Using 18-PET-FDG helped to identify a hypermetabolic mediastinal adenopathy, with pathology reporting non-caseating granulomas, findings suggestive of sarcoidosis. This reflects its usefulness for the diagnosis and staging of the disease, especially in situations where there is no evidence from other imaging studies of extra-neural disease.

Neurosarcoidosis Associated with Psychiatric Symptoms: Case Report

Background Sarcoidosis is a granulomatous disease predominant in women and black men that has inflammatory origin of unknown etiology; neurosarcoidosis is a rare and critical presentation of the disease. Case description A 26-year-old black female presented frontal headache for 1 year, as well as behavioral and mood changes for 15 days. Skull tomography and nuclear magnetic imaging of the skull revealed damaged meninges, a right frontal bone lesion, and an intraparenchymal contrast-enhancing lesion. Screening with computed tomography (CT) scans was performed, and it showed signs of bronchiectasis in the lower third of the right lung, but it was asymptomatic. The biopsy showed signs of reactional lesion with the presence of non-caseating granulomas. After the treatment with corticosteroids, the patient presented progressive improvement. Conclusions Neurosarcoidosis is a rare and critical pathology of sarcoidosis that presents a lytic bone lesion and clinical psychiatric symptoms; neurosarcoidosis is also rare in the literature.

Introdução A sarcoidose é uma doença granulomatosa de origem inflamatória de etiologia desconhecida, predominante em mulheres e negros, sendo a neurosarcoidose uma apresentação da doença rara relacionada a gravidade. Relato de Caso Paciente com 26 anos, negra, apresenta quadro cefaleia frontal ha 1 ano e há 15 dias apresentando sinais de alterações de comportamento e humor. Tomografia de crânio e Ressonância nucelar magnética de crânio que evidenciaram lesão óssea frontal direita, comprometimento meninges e lesão intraparenquimatosa captante de contraste. Realizado screening com tomografias que evidenciaram sinais de bronquiectasia em terço inferior pulmão direito, assintomática. Biopsia indicando sinais de lesão reacional com presenças de granulomas não caseosos, Após a manutenção da corticoterapia, a paciente apresentou melhora progressiva. Conclusões A neurosarcoidose é uma patologia grave e rara da sarcoidose, nesse caso apresenta uma lesão óssea lítica e sintomas psiquiátricos, apresentação rara na literatura.

Mielitis transversa longitudinal extensa: similares presentaciones, diferentes etiologías / Longitudinal transverse myelitis: similar presentations, different etiologies

La mielitis transversa longitudinal extensa (MTLE) se define como lesión de la médula espinal con una extensión de tres o más cuerpos vertebrales. La presentación clínica dependerá del área anatómica afectada incluyendo paraparesia, paraplejia, parestesias o pérdida sensorial en diferentes modalidades, síntomas urinarios entre otros. Sus etiologías son diversas como ser procesos metabólicos, tóxicos, inflamatorios, infecciosos. En el presente artículo describimos tres interesantes casos clínicos de MTLE. El primero una paciente joven con neuromielitis óptica; el segundo una paciente con neurosarcoidosis y el último caso un paciente con mielopatía tóxica producida por abuso de óxido de nitrógeno. Todos ellos con presentación clínica y radiológica similar resaltando la importancia de la obtención de una buena historia clínica.

Longitudinal extensive transverse myelitis (LETM) is defined as a spinal cord lesion that compromise 3 or more vertebral segments. Clinical presentation varies and will depend on the anatomical area that is compromised including paraparesis, paraplegia, paresthesia or sensory loss of any modalities and urinary or bowel symptoms. The etiologies are several such as toxic - metabolic abnormalities, inflammatory or infectious. Here in we present three interesting cases of LETM; the first case is a young patient with neuromyelitis optica; the second a case of neurosarcoidosis and the last case a toxic myelopathy secondary to nitrous oxide abuse. All of them with similarities in their clinical and radiologic presentation but with different etiologies highlighting the importance of obtaining a good medical history.

Spinal Cord Neurosarcoidosis after Cervical Compressive Myelopathy

Sarcoidosis is a multisystem noncaseating granulomatous disease that usually involves the respiratory system. It can involve any part of the central nervous system, but spinal-cord involvement is extremely rare. There have been a few of reports on the coexistence of compressive myelopathy and spinal-cord sarcoidosis, and compressive myelopathy may be associated with the development of inflammatory granuloma in spinal-cord sarcoidosis. We report a 65-year-old man who presented with gait disturbance due to spinal-cord sarcoidosis after compressive myelopathy.

Solitary Cavernous Sinus Neurosarcoidosis Mimicking Neurosyphilis

A differential diagnosis between neurosarcoidosis and neurosyphilis is particularly problematic in patients with a positive serologic result for syphilis. We report here a patient with a solitary cavernous sinus sarcoidosis who had a history of syphilis and showed rapidly progressing cavernous sinus syndrome. A transsphenoidal biopsy was performed and a histopathologic examination revealed a non-caseating granuloma with an asteroid body. His facial pain disappeared after steroid therapy. He received oral prednisolone for one year. A follow-up magnetic resonance imaging of the brain revealed resolution of the mass over the cavernous sinus. Particularly in patients with a history of syphilis, neurosyphilis should be included in a differential diagnosis of neurosarcoidosis.

Neurosarcoidosis after Uveitis

No abstract available.

Rapidly progressive dementia due to neurosarcoidosis / Demência rapidamente progressiva causada por neurosarcoidose

Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. METHODS: We report two cases of neurosarcoidosis presenting as RPD. RESULTS: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. CONCLUSION: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

Demência rapidamente progressiva (DRP) é tipicamente definida como um declínio cognitivo que progride para prejuízo funcional severo em menos de 1-2 anos, geralmente em semanas a meses. O diagnóstico rápido e acurado é fundamental, já que muitas condições que levam a DRP são reversíveis. MÉTODOS: Relatamos dois casos de neurosarcoidose que se apresentaram como DRP. RESULTADOS: Caso 1: Uma mulher de 61 anos desenvolveu uma DRP associada a perda de acuidade visual. Em sete meses evoluiu com dependência para auto-cuidado. A ressonância magnética (RM) revelou lesões encefálicas temporais e supraselares. Evoluiu com melhora parcial após tratamento com metilprednisolona intravenosa em altas doses. Caso 2: Uma mulher de 43 anos que estava em tratamento para diabetes insipidus desenvolveu uma amnésia episódica severa um ano após o início dos sintomas cognitivos. A RM anterior mostrava uma lesão hipotalâmica, e ela recebeu tratamento oral com prednisona e ciclofosfamida. Houve redução dos achados da RM, porem sem melhora dos déficits cognitivos. A biópsia cerebral mostrou granulomas não caseosos e angeíte granulomatosa; o tratamento foi modificado para metilprednisolona intravenosa em altas doses, com resposta sintomática pobre. CONCLUSÃO: O diagnóstico de DRP por neurosarcoidose pode ser desafiador quando a doença está restrita ao sistema nervoso central. Nestes casos, a apresentação clínica da DRP associada a disfunção neuroendócrina e visual, exames de imagem com lesões hipotalâmicas e, em alguns casos, a biópsia cerebral são fundamentais para um diagnóstico correto. é possível que o diagnóstico e tratamento precoces poderiam ter trazido melhores resultados nesses pacientes.

Neurosarcoidosis: guidance for the general neurologist / Neurosarcoidose: orientações para o neurologista geral

Neurosarcoidosis (NS) more commonly occurs in the setting of systemic disease. The diagnosis is based on a clinical history suggestive of NS, presence of noncaseating granulomas, and supportive evidence of sarcoid pathology, laboratory, and imaging studies. NS could involve any part of the nervous system and often demands high doses of steroids for symptom control. It presents low response to isolated steroids administration and frequently requires immunosuppressive agents. In NS, lymphocytes are polarized toward an excessive Th1 response, leading to overproduction of TNF-alpha and INF-gama, as well as lL-2 and IL-15. Infliximab, a chimeric monoclonal antibody that neutralizes the biological activity of TNF-alpha, is a new option in the NS treatment. We revised pathophysiology, clinical manifestations, diagnostic work up, and treatment of NS as guidance for the general neurologist.

A neurosarcoidose (NS) ocorre frequentemente no contexto de doença sistêmica. O diagnóstico é baseado na história clínica sugestiva de NS, presença de granulomas não-caseosos e achados anatomopatológicos, laboratoriais e radiológicos de sarcoidose. A NS causa manifestações neurológicas variadas, que apresentam, em geral, baixa resposta ao corticoide isoladamente e, portanto, necessitam uso de imunossupressores. Na NS, os linfócitos estão polarizados para resposta Th1 excessiva, levando à produção aumentada de TNF-alfa e IFN-gama, assim como IL-2 e IL-15. Infliximabe, um anticorpo monoclonal quimérico que neutraliza a atividade biológica do TNF-alfa, é uma nova opção no tratamento da NS. Revisou-se a fisiopatologia, as manifestações clínicas, o diagnóstico e o tratamento da NS para orientar neurologistas gerais.

Treatment of Hydrocephalus Associated with Neurosarcoidosis by Multiple Shunt Placement

A 31-year-old man was admitted to our hospital due to hydrocephalus with neurosarcoidosis. Ventriculo-peritoneal shunting was performed in the right lateral ventricle with intravenous methylprednisolone. Subsequently, after 4 months, additional ventriculo-peritoneal shunting in the left lateral ventricle was performed due to the enlarged left lateral ventricle and slit-like right lateral ventricle. After 6 months, he was re-admitted due to upward gaze palsy, and magnetic resonance image showed an isolated fourth ventricle with both the inlet and outlet of fourth ventricle obstructed by recurrent neurosarcoidosis. Owing to the difficulty of using an endoscope, we performed neuronavigator-guided ventriculo-peritoneal shunting via the left lateral transcerebellar approach for the treatment of the isolated fourth ventricle with intravenous methyl prednisolone. The patient was discharged with improved neurological status.

Isolated Spinal Cord Neurosarcoidosis Diagnosed by Cord Biopsy and Thalidomide Trial

We report a case of 54-yr-old woman who presented with 4-extremities weakness and sensory changes, followed by cervical spinal cord lesion in magnetic resonance imaging. Based on the suspicion of spinal tumor, spinal cord biopsy was performed, and the histology revealed multinucleated giant cells, lymphocytes and aggregated histiocytes within granulomatous inflammation, consistent with non-caseating granuloma seen in sarcoidosis. The patient was treated with corticosteroid, immunosuppressant and thalidomide for years. Our case indicates that diagnosis of spinal cord sarcoidosis is challenging and may require histological examination, and high-dose corticosteroid and immunosuppressant will be a good choice in the treatment of spinal cord sarcoidosis, and the thalidomide has to be debated in the spinal cord sarcoidosis.

Hydrocephalus as a Presenting Manifestation of Neurosarcoidosis : Easy to Misdiagnose as Tuberculosis

We present a case of hydrocephalus as the primary manifestation of neurosarcoidosis. Sarcoidosis is a rare disease in Korea and its incidence is much lower than that of tuberculosis. Diagnosis is made by pathologic findings and by exclusion of other granulomatous disorders. Neurosarcoidosis is observed in approximately 5% of sarcoidosis. Its common manifestations are facial palsy (50% of patients with neurosarcoidosis) and optic neuritis. Hydrocephalus is a very uncommon reported finding. Although the typical presentation of sarcoidosis such as facial palsy is not a diagnostic dilemma, more atypical presentations such as hydrocephalus with altered mentality in a tuberculosis patient can lead to a misdiagnosis.

Moyamoya-Like Vasculopathy in Neurosarcoidosis

A 31-year-old man presented with dull headache and memory disturbance lasting for one week. Computed tomographic scans revealed acute hydrocephalus. The cerebrospinal fluid contained 53 leukocytes/mm3, with a mononuclear preponderance and no erythrocytes. Magnetic resonance imaging revealed hydrocephalus and leptomeningeal enhancement. Magnetic resonance angiography and digital subtraction angiography showed supraclinoid occlusion of the right internal carotid artery, which resembled unilateral moyamoya disease. Neuroendoscopic biopsy of a lesion in the septum pellucidum revealed noncaseating granulomas, which was consistent with sarcoidosis. The patient was successfully managed with intravenous methylprednisolone and ventriculoperitoneal shunting. To our knowledge, this is the first case of moyamoya-like vasculopathy associated with neurosarcoidosis.

A Case of Pulmonary Sarcoidosis Combined with Neurosarcoidosis / 결핵및호흡기질환

Sarcoidosis is a multi-systemic syndrome of an unknown etiology, and it is characterized by the formation of multiple noncaseating granulomas that disrupt the architecture and function of the tissues in which they reside. The most commonly affected organs are lung, skin and lymph nodes. Overt clinical involvement of the nervous system is uncommon and this occurs in about 5% of all patients during the course of their disease. The most common manifestations are granulomatous leptomeningitis, cranial nerve palsy, electrolyte or other endocrinologic abnormalities, but isolated memory impairment is a rare manifestation. This is a case of 59 years-old male with recent memory impairment, and he was previously diagnosed with pulmonary sarcoidosis by transbronchial lung biopsy. The brain MRI imaging revealed the leptomeningeal and parenchymal involvement of sarcoidosis. He was treated with high dose corticosteroid and his memory function was improved to nearly a normal level. We report here on a case of successful treatment of pulmonary sarcoidosis combined with neurosarcoidosis with using high dose corticosteroid, and the patient presented with recent memory impairment.

A Case of Possible Neurosarcoidosis with Dementic Features

No abstract available.

Neurosarcoidosis: First reported case in Thailand.

Sarcoidosis is a chronic multisystem granulomatous disease of unknown aetiology, which has a propensity to affect the lungs and rarely the nervous system; neurosarcoidosis may be an enigmatic diagnosis which is often entertained but rarely made with conviction. Prevalence rates for intrathoracic sarcoidosis vary from greater than 50 per 100, 000 e.g. in New York African-Americans, to under 10 per 100,0002. Much higher prevalence rates were obtained when consecutive postmortems were performed on approximately 60% of all deaths in an area of Sweden, of known to have sarcoidosis during life, yielding a prevalence of 641 per 100,0003. Previous data from large series of patients with sarcoidosis have estimated that approximately 5% of such patients will have clinical involvement of the nervous system4-7, although post-mortem studies suggest that ante-mortem diagnosis is only made in 50% with nervous system involvement. Therefore one can estimate that about 5 to 10 patients per million population will have clinical neurosarcoidosis. In Thailand there has been no previous report of neurosarcoidosis, thus our patient is the first documented case in the Kingdom.