Uveo-Meningitis and Myelodysplastic Syndrome.

Uveo-meningeal syndromes are any disorder characterized by the involvement of the uvea and the meninges. They can have multiple causes, including infectious, autoimmune and malignant diseases. We report the case of a patient with a 10-year-old diagnosis of myelodysplastic syndrome that had been stable. He presented with new onset uveitis, ataxia, diplopia and fluctuating consciousness level, without any obvious cause revealed by brain imaging or blood chemistries. An extensive aetiological search showed no obvious cause. Initially, the patient improved spontaneously and was discharged. However, 2 months later, he deteriorated once again, this time with disperse adenomegalies that were not present previously. The bone marrow biopsy showed a high number of blasts, which affirmed the progression of the previously known myelodysplastic syndrome. The lymph nodes were not biopsied due to very low life expectancy, making the procedure futile. A presumptive diagnosis of uveo-meningitis caused by paraneoplastic syndrome was made. The patient died a few months later. LEARNING POINTS: Uveo-meningeal syndrome (UMS) is a rare entity usually of infectious or inflammatory aetiology.UMS may be caused by some neoplastic disorders, mainly lymphoma and metastasis of solid tumours; myelodysplastic syndrome (MDS) has not been previously implicated.Diagnosis should include lumbar puncture and a thorough search for the aetiology; treatment is directed towards the aetiology.

Uveo-meningeal syndrome secondary to Herpes Simplex Virus related acute retinal necrosis.

PURPOSE: To present a rare case of uveo-meningeal syndrome secondary to herpes simplex virus (HSV-1) in a patient with acute retinal necrosis. OBSERVATIONS: A 49-year-old female with a past medical history of herpes simplex encephalitis 18 years prior presented with a 3-day history of right sided headache and decreased vision of the right eye. Her visual acuity was 20/30 in the right eye and 20/20 in the left eye. Clinical examination revealed right relative afferent pupillary defect, panuveitis, and retinal necrosis. Examination of the left eye was unremarkable. Cerebral spinal fluid (CSF) analysis by polymerase chain reaction (PCR) was negative for herpes simplex virus 1 (HSV-1) but did reveal pleocytosis consistent with meningitis. The patient was admitted and empirically treated with intravenous acyclovir (10 mg/kg every 8 hours) and systemic steroids. Topical steroids and cycloplegia were also started. Magnetic resonance imaging revealed no leptomeningeal, pachymeningeal, or parenchymal enhancement. Systemic autoimmune and infectious workup were unremarkable. Based on clinical exam findings and negative PCR results, an anterior chamber tap was performed with aqueous fluid PCR testing which revealed 71,000 copies of HSV-1. A repeat lumbar puncture was performed on day three of admission and revealed a decrease in pleocytosis after initiation of acyclovir therapy and remained negative for HSV on PCR testing. She was discharged home on intravenous acyclovir, topical steroids, and topical cycloplegics. Her retinal necrotic lesions continued to regress and her headaches continued to improve. CONCLUSIONS AND IMPORTANCE: Uveo-meningeal syndromes are a rare clinical entity that involve the uvea, retina, and meninges. This case highlights the importance of aqueous fluid PCR testing despite negative CSF PCR, as it may hasten treatment with antiviral therapies to preserve vision and limit neurologic sequelae.

Two Cases of Uveo-meningeal Syndrome

PURPOSE: We report two cases of uveo-meningeal syndrome involving the retina, uvea, and optic disc in both eyes after viral meningitis. CASE SUMMARY: A 16-year-old female was referred to our department with blurred vision in both eyes. She was hospitalized in the pediatric ward with viral meningitis. She showed a norma best-corrected visual acuity (BCVA) and normal intraocular pressure in both eyes, but had severe inflammation in the anterior chamber on slit lamp examination, and optic disc edema and multiple whitish lesions on fundus examination. She was treated with intravenous antibiotic injections and steroid eye drops. After close observation, inflammation in the anterior chamber, optic disc edema, and the multiple whitish lesions in the retina were improved. A 27-year-old male who was treated for viral meningitis at the neurology department was referred to us with blurred vision in both eyes. His BCVAs were 0.7 (right eye) and 0.6 (left eye). The intraocular pressure was normal in both eyes. Slit lamp examination revealed inflammation in the anterior chamber and optic disc edema, and a fundus examination revealed multiple infiltrations. He received treatment for presumed herpes virus infection. After close observation, inflammation in the anterior chamber, optic disc edema, and multiple infiltrations with hemorrhage in the retina were improved. CONCLUSIONS: Clinicians should consider the possibility of uveo-meningeal syndrome, which can cause inflammation in the uvea,retina, and optic disc simultaneous with viral meningitis accompanying blurred vision.

Aspergillosis of the central nervous system in a previously healthy patient that simulated Creutzfeldt-Jakob disease.

BACKGROUND: The presence of Aspergillus in the central nervous system (CNS) is rare in immunocompetent patients but not in immunocompromised patients who may have a more common infection. This article describes a case of an adult immunocompetent patient with a diagnosis of cerebral aspergillosis and with a clinical process of rapidly progressive dementia which simulated a Creutzfeldt-Jakob syndrome. CASE DESCRIPTION: A 34-year-old adult was previously healthy and had no medical history of any significance. The patient had suffered only facial trauma 8 months before admission. One month prior to admission, he showed rapidly progressing changes in his behavior and higher mental functions. He was admitted to the emergency room with an occipital headache with 2 months of history. By the time he arrived, he suffered from total disability and was prostrate. He was diagnosed with meningeal and demential syndrome in the process of being studied. After starting the diagnostic approach by investigating cerebrospinal fluid, a magnetic resonance of the skull, an electroencephalogram, a brain biopsy was indicated. The histopathological study reported the presence of the hyphae characteristics of Aspergillus. The patient died 7 days after the diagnosis. CONCLUSION: Cerebral aspergillosis is a common aggressive disease in immunosuppressed patients. However, the disease is rare in individuals with respected immunity and in individuals with neurological impairment and a rapid and progressive deterioration of mental functions. The suspected diagnosis should always be considered given its poor prognosis and the encouraging efficacy of antifungal treatment administered in a timely manner.

Síndrome meningeo como manifestación de apoplejia hipofisiaria, análisis prospectivo de siete casos / Meningeal syndrome as manifestation of pituitary apoplexy, prospective analysis of seven cases

Introducción: la apoplejía hipofisiaria es una grave pero poco frecuente emergencia médico -neuroquirúrgica, con una incidencia global reportada del 1 por ciento a 2 por ciento Es causado por una hemorragia o infarto en relación a la glándula pituitaria, pudiendo existir extravasación de contenido necrótico o hemorrágico al espacio subaracnoideo, manifestándose como un síndrome meníngeo aséptico o hemorrágico. Sin embargo, su frecuencia no está estudiada. Material y Métodos: Se realizó un registro prospectivo entre enero de 2013 y agosto de 2014, para el estudio de líquido céfalo raquídeo, en pacientes con diagnóstico clínico- imagenológico de apoplejía hipofisiaria. Además un registró detallado de las manifestaciones clínicas y de laboratorio. Resultados: En este período se reclutaron 8 casos con apoplejía hipofisiaria clínica, de los cuales 7 fueron incluidos, siendo excluido un paciente por rechazar su participación en el estudio. De los 7 pacientes restantes, se evidenciaron signos meníngeos clínicos en el 86 por ciento (6/7), confirmando alteraciones del estudio cito-químico en todos ellos. En cada caso se descartó patología infecciosa o vascular como etiología. Conclusión: Si bien el debut de una apoplejía hipofisiaria como un síndrome meníngeo aséptico o hemorragia subaracnoidea, se encuentra documentado como casos anecdóticos, nuestros resultados apuntan a que sería una manifestación frecuente e importante a considerar para un adecuado diagnóstico diferencial y monitoreo de complicaciones infrecuentes.

Introduction: pituitary apoplexy is a serious but rare neurosurgical emergency, with an overall reported incidence of 1 percent to 2 percent. It is caused by bleeding or infarction related to the pituitary gland, there may be necrotic or hemorrhagic extravasation content to the subarachnoid space, manifesting as an aseptic o hemorrhagic meningeal syndrome. However, their frequency is not studied. Material and Methods: A prospective registry between January 2013 and August 2014, for the study of cerebro spinal fluid in patients with clinical and imaging diagnosis of pituitary apoplexy was performed. In addition, a detailed analysis of the clinical sintoms and laboratory was recorded. Results: In this period, 8 cases with clinical pituitary apoplexy were recluted, of which 7 were included, being excluded from a patient who refuses to participate in the study. Of the remaining 7 patients,clinical meningeal signs were evident in 86 percent (6/7), confirming alterations cyto-chemical study all of them. In each case infectious or vascular pathology was ruled out as a cause. Conclusion: While the debut of a pituitary apoplexy as an aseptic meningeal syndrome or subarachnoid hemorrhage, is documented as anecdotal cases, our results would suggest that is a common and important manifestation, to consider an appropriate differential diagnosis and monitoring of rare complications.

Variabilidad en las manifestaciones clínicas de la meningoencefalitis por tuberculosis / Variability in the clinical manifestations of meningeal encephalitis due to tuberculosis

Con el objetivo de describir las manifestaciones clínicas de pacientes con meningoencefalitis por tuberculosis (TBC), se realizó un estudio descriptivo, retrospectivo mediante la revisión de historias clínicas de 15 pacientes adultos, evaluados por el servicio de Neurología en el SAHUM durante el periodo de Enero 2002 a Diciembre 2008. Se analizó: edad, sexo, procedencia, manifestaciones clínicas, tiempo entre el inicio de los síntomas y la hospitalización y tratamiento. De los 15 pacientes, 10 (66,67%) fueron de sexo femenino, 9 (60,0%) entre 20 y 29 años de edad. El 53,34% eran procedentes de los Municipios Mara y Páez. Las manifestaciones clínicas fueron; cefalea y rigidez de nuca 14 (93,33%), alteración de la consciencia 13 (86,67%), alteración mental 7 (46,66%), convulsión 8 (53,33%), hipertensión intracraneana 5 (33,33%), déficit Motor 3 (20,00%), alteración de pares craneales 2 (13,33%) como falso signo localizador y en 2 (13,33%) diplopía. La citoquímica del líquido cefalorraquídeo reveló hipoglucorraquia en el 100,00% de los pacientes e hiperproteinorraquia. La coloración de Ziehl Neelsen fue positiva en 1 (6,67%). 6 pacientes (40,00%) tenían más de 1 mes con TBC. Se concluye que son diversas las manifestaciones clínicas de meningoencefalitis por TBC, siendo más frecuente el síndrome meníngeo

With the objective of describing clinical manifestations in patients with meningeal encephalitis due to tuberculosis (TBC), a descriptive, retrospective study was made by reviewing clinical histories of 15 adult patients with a diagnosis of TBC in the SAHUM neurology service from January 2002 to December 2008. Age, sex, clinical manifestations, hospitalization and treatment were analyzed. Of the 15 patients, 10 (66.67%) were female, 9 (60.0%) were between 20 and 29 years of age; 53.34% came from the Mara and Páez Municipalities. The clinical manifestations were: headache and nape of the neck rigidity, 14 (93.33%); alteration of consciousness, 13 (86.67%); alteration of mental state, 7 (46.66%); convulsions, 8 (53.33%); intracranial hypertension, 5 (33.33%); motor deficit, 3 (20.00%); alteration of cranial pairs, 2 (13.33%) as a false locator sign and diplopia, 2 (13.33%). Cytochemical study of the cerebrospinal fluid revealed hypoglycorrhachia in 100.00% of the patients. Ziehl Neelsen coloration was positive in 1 (6.67%). Six patients (40.00%) had had the disease for more than 1 month. Conclusions were that the clinical manifestations of meningeal encephalitis due to TBC are diverse, with the meningeal syndrome as the most frequent