Gluten and Neuroimmunology. Rare association with Myasthenia Gravis and Literature Review

SUMMARY As the celiac disease (CD), the non-celiac gluten sensitivity (NCGS) has also been associated with several autoimmune manifestations. It is rarely associated with myasthenia gravis (MG). This paper shall introduce the case of a young female patient, initially presenting a peripheral neuropathy framework. During clinical and neurological follow-up, she began to present symptoms of various immune-mediated morbidities. Diseases related to gluten represent a clinical spectrum of manifestations with a trigger in common, the ingestion of gluten. CD is the most well-known and serious disease of the spectrum, also called gluten-sensitive enteropathy. The NCGS is diagnosed from clinical evidence of improvement in symptoms followed by a Gluten Free Diet (GFD) in patients without signs of enteropathy in duodenal biopsy. There are indications that, although rare, with a prevalence of 1 in 5000, myasthenia gravis (MG) may occur more often when CD is also present. Between 13 to 22% of the patients with MG have a second autoimmune disorder. However, it is often associated with dermatomyositis or polymyositis, lupus erythematosussystemic lupus erythematosus, Addison's disease, Guillain-Barré syndrome and juvenile rheumatoid arthritis. Thus, the symptoms of neuromuscular junction involvement may give a diagnostic evidence of this rare association.

Non-progressive cerebellar ataxia and previous undetermined acute cerebellar injury: a mysterious clinical condition / Ataxias cerebelares não-progressivas e lesão cerebelar aguda prévia indeterminada: uma condição clínica misteriosa

Cerebellar ataxias represent a wide group of neurological diseases secondary to dysfunctions of cerebellum or its associated pathways, rarely coursing with acute-onset acquired etiologies and chronic non-progressive presentation. We evaluated patients with acquired non-progressive cerebellar ataxia that presented previous acute or subacute onset. Clinical and neuroimaging characterization of adult patients with acquired non-progressive ataxia were performed. Five patients were identified with the phenotype of acquired non-progressive ataxia. Most patients presented with a juvenile to adult-onset acute to subacute appendicular and truncal cerebellar ataxia with mild to moderate cerebellar or olivopontocerebellar atrophy. Establishing the etiology of the acute triggering events of such ataxias is complex. Non-progressive ataxia in adults must be distinguished from hereditary ataxias.

Ataxias cerebelares representam um grupo amplo de doenças neurológicas secundárias a disfunções cerebelares ou das vias associadas, raramente cursando com etiologias adquiridas de início agudo e com evolução crônica não-progressiva. Nós avaliamos pacientes com ataxia cerebelar adquirida não-progressiva com apresentação prévia aguda ou subaguda. Foi realizada caracterização clínica e de neuroimagem de pacientes adultos com ataxia adquirida não-progressiva. Cinco pacientes foram identificados com o fenótipo clínico de ataxia adquirida não-progressiva. A maior parte dos pacientes apresentou início juvenil ou no adulto, de forma aguda ou subaguda, de ataxia cerebelar appendicular e de tronco com atrofia cerebelar ou olivopontocerebelar leve a moderada. Estabelecer a etiologia dos eventos agudos desencadeantes de tais ataxias é complexo. Ataxia não-progressiva em adultos deve ser diferenciada das ataxias hereditárias.

patterns of clinical presentations of cerebellar syndromes among adult Sudanese patients

Cerebellar syndromes are one of the commonest neurological diseases. To study the patterns of clinical presentations of cerebellar syndromes and to identify the possible causes. This is a prospective hospital based, cross-sectional study. One hundred adult Sudanese patients with cerebellar syndromes were included in the study during the period from January 2006 -January 2007. The most common age group affected was 18-25 years. Male to female ratio was 1.5: 1; unsteadiness on walking was the most common symptom [83%]. Gait-ataxia was the most common sign [83%]. Cerebrovascular disease was the most common aetiology [25%]. Cerebellar syndromes are not rare in Sudan. However, they were diagnosed more commonly at the central regions of the country probably because of more awareness of patients and better facilities for diagnosis. The age of onset, the male predominance, the presentation and clinical findings were not different from reported literature. This also goes for the common causes apart from alcohol which is a strikingly rare as a cause in this study and could be accounted for the implementation of Elshariya [Islamic laws] Laws in Sudan

Infarto pseudotumoral del cerebelo / Malignant cerebellar infarction

El diagnóstico de infarto cerebeloso en su presentación inicial puede ser difícil, su reconocimiento tardío puede asociarse a graves complicaciones. Aunque sólo representa 2 por ciento a 3 por ciento de los infartos encefálicos, afecta a un importante número de pacientes, muchos de los cuales son jóvenes. De todos los infartos de cerebelo sólo 10 por ciento evolucionará en forma maligna, siendo denominado infarto pseudotumoral de cerebelo. Las causas más habituales son la embolia, la disección de la arteria vertebral y la aterotrombosis. La arteria cerebelosa póstero-inferior es la más frecuentemente comprometida, y en un tercio de los casos se encuentra una fuente cardioembólica. Su rasgo distintivo es el efecto de masa, el mismo que ocasiona compresión de troncoencéfalo e hidrocefalia aguda, generando un deterioro cuantitativo de conciencia. Las neuroimágenes, tomografía computada y resonancia magnética de encéfalo, son de vital importancia para establecer la presencia de un infarto cerebeloso e identificar potenciales complicaciones. Es importante recordar que la tomografía computada es menos sensible que la resonancia magnética para establecer el diagnóstico. El manejo especializado multidisciplinario y la implementación de las medidas de soporte generales y específicas pueden mejorar las posibilidades de sobrevida y recuperación funcional. Ante la presencia de un deterioro de conciencia, un abordaje quirúrgico agresivo pareciera ser la mejor opción de tratamiento.

The diagnosis of cerebellar infarction at initial presentation can be difficult, delayed recognition can be associated with serious complications. Although representing only 2 percent to 3 percent of brain infarcts, affects a significant number of patients, many of whom are young. Only 10 percent of cerebellar infarcts evolve into malignant form, being named pseudotumoral cerebellar infarction. Common causes include embolism, vertebral artery dissection and atherothrombosis. The postero-inferior cerebellar artery is the most frequently committed, and one third of cases there is a cardioemboIic sourse. Its distinguishing feature is the mass effect, causing brain stem compression and acute hydrocephalus, and generating a quantitative impairment of consciousness. The brain imaging, computed tomography and magnetic resonance imaging of brain, are of vital importance to establish the presence of a cerebellar infarct and identify potential complications. It is important to remember that computed tomography is less sensitive than magnetic resonance for diagnosis. The multidisciplinary specialized management and implementation of measures of general and specific support can improve the chances of survival and functional recovery. In the presence of impaired consciousness, an aggressive surgical approach appears to be the best treatment option.

The Patterns of Clinical Presentations of Cerebellar Syndromes among Adult Sudanese Patients

Cerebellar syndromes are one of the commonest neurological diseases.Objectives:To study the patterns of clinical presentations of cerebellar syndromes and to identify the possible causes.Methods:This is a prospective hospital based; cross-sectional study. One hundred adult Sudanese patients with cerebellar syndromes were included in the study during the period from January 2006 January 2007.Results:The most common age group affected was 18 - 25 years.Male to female ratio was 1.5:1unsteadiness on walking was the most common symptom (83).Gait-ataxia was the most common sign (83). Cerebrovascular disease was the most common etiology (25).Conclusion:Cerebellar syndromes are not rare in Sudan.However; they were diagnosed more commonly at the central regions of the country probably because of more awareness of patients and better facilities for diagnosis.The age of onset; the male predominance; the presentation and clinical findings were not different from reported literature.This also goes for the common causes apart from alcohol which is a strikingly rare as a cause in this study and could be accounted for the implementation of Elshariya (Islamic laws) Laws in Sudan

Intracranial complications of chronic suppurative otitis media

To study the presentations of various intra cranial complications of chronic suppurative otitis media [SOM]. This prospective and descriptive study was conducted in the department of ENT, Lady Reading Hospital Peshawar from April 2006 to march 2007. All the cases with suspected intra cranial complications were admitted to the department and assessed clinically and radiologically. Audiological, and laboratory investigations were done as well. Out of 50 cases 36 were male and 14 female ranging from 10-40years in age. Complications like meningitis and brain abscesses were more common in males in the 2[nd] and 3[rd] decade of life [72%]. Most of the complications were from atticoantral diseases. The main presenting features were foul smelling otorrhea, headache, and fever, neck stiffness, chloestosteatoma and granulations in the ear. Meningitis [46%], temporal lobe abscess [36%] and extra dural abscess [14%] were the commonest complications. Burr hole aspiration, for intra cranial abscess and radical/modified radical mastoidecomy for SOM were the main surgical procedure carried out for these patients. Otogenic intra cranial complications like meningitis and brain abscesses are still common in spite of advances in the medical sciences. Complications are common in 2[nd] and 3[rd] decade of life. Meningitis is the commonest complication followed by brain abscess Burr whole aspiration with modified, radical mastoidectomy is the main stay of treatment along with parenteral antibiotics

Congenital Cerebellar Mixed Germ Cell Tumor Presenting with Hemorrhage in a Newborn

We report here on a neonate with congenital cerebellar mixed germ cell tumor, and this initially presented as cerebellar hemorrhage. Postnatal cranial ultrasonography revealed an echogenic cerebellar mass that exhibited the signal characteristics of hemorrhage rather than tumor on MR images. The short-term follow-up images also suggested a resolving cerebellar hemorrhage. One month later, the neonate developed vomiting. A second set of MR images demonstrated an enlarged mass that exhibited changed signal intensity at the same site, which suggested a neoplasm. Histological examination after the surgical resection revealed a mixed germ cell tumor.

Cerebellar hemorrhage after supratentorial surgery for treatment of epilepsy: report of two cases.

Hemorrhage occurring at regions remote from the operative site is an infrequent complication. Although the mechanism remains unclear, previous reports implicate over drainage of cerebrospinal fluid as the predominant mechanism. The authors report two cases of cerebellar hemorrhage after supratentorial surgery. Two young patients underwent left hemispherectomy and fronto-temporal resection for the treatment of refractory hemispheric and multiregional epilepsy. The hemorrhage manifested early in the immediate postoperative period as delayed awakening. The diagnosis was established by computed tomography. Treatment consisted in external ventricular drainage in case 1 and conservative treatment in case 2. Both patients recovered without major neurological deficits. Early detection and awareness of this complication may help to avoid further neurological morbidity and mortality.

Infarto cerebelar: análise de 151 pacientes / Cerebellar infarction: analysis of 151 patients

Este estudo apresenta o tratamento de 151 pacientes com infarto cerebelar, sendo 98 homeNs (65 por cento) e 53 mulheres (35 por cento), com média de idade de 62,4 anos. Hidrocefalia obstrutiva foi diagnosticada em 7,9 por cento dos pacientes associada com um infarto cerebelar extenso e em todos os 11 pacientes operados (7,2 por cento). Quatro pacientes foram submetidos a derivação ventricular externa com 3 óbitos (75 por cento) e 7 foram submetidos a craniectomia descompressiva suboccipital com 2 óbitos (28,5 por cento). A mortalidade no grupo clínico foi de 15 pacientes (10,7 por cento). Vertigem, vômito, sinal de Romberg e dismetria foram os sinais e sintomas de envolvimento cerebelar mais frequentemente observados. Infarto cerebelar devido a embolismo provocado por cirurgia cardiovascular ocorreu em 57 pacientes (37,7 por cento).Infarto cerebelar como fato isolado ocorreu em 59 pacientes (39 por cento) e infartos cerebelares associados a infartos de outras regiões ocorreram em 92 pacientes (61 por cento). A ressonância magnética foi o melhor método para o diagnóstico das lesões, embora a tomografia pôde mostrar infarto cerebelar em 68 pacientes (78 por cento).

Herniation of the cerebellar tonsils after suprasellar arachnoid cyst shunt: case report

Sabe-se que o deslocamento caudal das tonsilas cerebelares pode ocorrer em associação com condições clínicas tais como: lesão expansiva intracraniana ou malformação de Chiari I e II. Pode ainda ser adquirido após repetidas punções lombares ou lomboperitoniostomia. A ocorrência de herniação cerebelar após derivação de cisto aracnóide intracraniano é evento extremamente raro, existindo apenas três casos relatados na literatura médica. O caso de menino de 9 anos de idade, com puberdade precoce e cisto aracnóide supra-selar, que desenvolveu herniação sintomática das tonsilas cerebelares três anos após cistoperitoniostomia. O paciente foi submetido a craniectomia suboccipital com plástica dural e tonsilectomia parcial, apresentando remissão dos sintomas. Discutimos a patogênese sugerida na literatura.

Otogenic cerebellar abscess drained through mastoidectomy in a 14 year-old girl

A 14 year old girl, with a past history of chronic otitis media was presented with otogenic cerebellar abscess. CAT scan finding, and initial management of the patient are described. Radical Mastoidectomy was performed for the drainage of the abscess which was complemented with antibiotics and metronidazol therapy. A second operation for covering cerebellar hernia was performed. The outcome of the whole procedure was good the patient was-discharged after a hospital stay of 48 days

Cerebellar hemorrhage as a complication of temporal lobectomy for refractory medial temporal epilepsy: report of three cases

A hemorragia cerebelar faz parte das potenciais complicações dos procedimentos neurocirúrgicos. De forma geral, é considerada uma condição rara. Entretanto, há aparente propensão dos pacientes sub metidos ao tratamento cirúrgico de epilepsia em apresentar este tipo de complicação, quando compara dos com outros grupos cirúrgicos. O posicionamento da cabeça, excessiva drenagem de líquido cefalorraquidiano e a excisão de tecido cerebral não expansível (ou talvez combinações entre os três) constituem as po ten ciais causas da hemorragia cerebelar remota. Entre os 118 pacientes em nossa série de LTA » AH, identifi camos 3(2.5%) casos de hemorragia cerebelar. Relatamos os três casos desta natureza, com revisão da lite ratura pertinente a esta complicação.

Familial type IV hypertriglyceridemia presenting as hemiparesis with cerebellar signs.

Stroke in pediatric patients is distinctive as compare to adults. The authors report a rare case of familial hypertriglyceridemia type IV who had left hemiparesis with cerebellar signs. There was no history of oral trauma, head injury, convulsions, acute gastroenteritis, meningitis or otitis media.

Paradoxical embolism through patent foramen ovale causing cerebellar infarction in a young boy.

A previously healthy young boy who suffered an acute stroke involving superior cerebellar artery circulation is presented here. Echocardiography revealed a patent foramen ovale through which paradoxical embolism had probably occurred. Low dose aspirin was started and surgical closure was planned to prevent further recurrences.

Abscesso cerebelar tratado clinicamente: relato de caso / Cerebellar abscess treated by clinical management: case report

Apresentamos um caso de abscesso cerebelar secundário a otite média crônica, localizado no hemisfério cerebelar direito, em uma paciente pediátrica, que foi tratado clinicamente. Obtendo resoluçäo sem necessidade de cirurgia. A antibioticoterapia foi escolhida segundo critérios da literatura para os agentes etiológicos mais prováveis. Discutimos a patogênese, histopatogênese, o quadro clínico, o diagnóstico e as formas de tratamento dos abscessos do sistema nervoso central

Deterioro cerebeloso inducido por aislamiento social temprano: estudio morfológico e inmunocitoquímico / Cerebellar impairment induced by early social isolation: a morphological and immunocytochemical study

En el presente trabajo se utiliza el modelo de aislamiento social en animales a objeto de estudiar el efecto que produce las experiencias tempranas adversas sobre el desarrollo morfofuncional de la corteza cerebelosa. Se utilizaron 103 ratas de la cepa Sprague-Dawley de 18 días de vida, las cuales fueron sepradas en dos grupos de estudio: (a) control (SC; 3- 4 ratas por jaula y (b) aislado (IC); estas últimas se colocaron en comportamientos individuales hasta los 32 días de edad (P32). En esta etapa, el 50 por ciento de los animales IC y SC fueron sacrificadas para su estudio neural; el resto de las ratas del grupo IC fue retirado de sus compartimientos individuales y reubicados en su entorno social normal hasta el día P62, realizándose el análisis neural respectivo. En ambas fases ontogenéticas (P32 y P62) se estudió el desarrollo dendrítico y la expresión de calbindina-D28k (CBD) en células de Purkinje vermianas. Los resultados obtenidos mostraron que la deprivación social y sensoriomotriz temprana altera el crecimiento dendríco en estrecha relación con una disminución del contenido intracelular de CBD. Además, la interacción social post-deprivación sólo logró recuperar la expresión de CBD, permaneciendo el deterioro estructural. Estos resultados indican que las experiencias postnatales adversas alteran el desarrollo morfológico y funcional de las neuronas de Purkinje vermianas cuando se emplea el modelo de aislamiento social