RESUMEN
ABSTRACT Purpose: To evaluate the prevalence, clinical characteristics, and types of optic nerve involvement in patients with ocular toxoplasmosis. Methods: For this retrospective cross-sectional study, we examined all patients with active ocular toxoplasmosis referred to our Uveitis Section during the last 12 years, and we included patients with optic nerve involvement in the study. The primary outcome was the prevalence of optic nerve involvement, and secondary outcomes included the types of optic nerve involvement and the final best-corrected visual acuity after treatment. Results: The prevalence of optic nerve involvement was 14.4%, with the leading cause being the activation of a juxtapapillary lesion (70.5%). We found papillitis in two eyes and neuroretinitis in two eyes (11.7% for each). We only detected one optic nerve involvement secondary to a distant active lesion (5.8%). Sixteen patients (94.1%) had unilateral ocular toxoplasmosis. The overall final best-corrected visual acuity after treatment was 10/10 (LogMAR = 0.0) excluding the three patients with a juxtapapillary scar involving the macula. Conclusions: Optic nerve involvement was common in patients with ocular toxoplasmosis. The main type of optic nerve involvement was caused by activation of an old juxtapapillary lesion. Treatment was quickly effective, but the best-corrected visual acuity was dependent on the presence of a scar in the papillomacular bundle.
RESUMO Objetivos: Avaliar a prevalência, características clínicas e tipos de acometimento do nervo óptico em pacientes com toxoplasmose ocular. Métodos: Para este estudo retrospectivo transversal, examinamos todos os pacientes com toxoplasmose ocular ativa encaminhados ao nosso Setor de Uveíte nos últimos 12 anos, e incluímos pacientes com comprometimento do nervo óptico no estudo. O resultado primário foi a prevalência do envolvimento do nervo óptico, e os resultados secundários incluíram os tipos de envolvimento do nervo óptico e a acuidade visual final melhor corrigida após o tratamento. Resultados: A prevalência de acometimento do nervo óptico foi 14,4%, sendo a principal causa a ativação de uma lesão justapapilar (70,5%). Encontramos papilite em dois olhos e neuroretinite em dois olhos (11,7% para cada um). Apenas detectamos um comprometimento do nervo óptico secundário a uma lesão ativa distante (5,8%). Dezesseis pacientes (94,1%) apresentavam toxoplasmose ocular unilateral. A acuidade visual final com melhor correção após o tratamento foi 10/10 (LogMAR= 0,0) excluindo os três pacientes com uma cicatriz justapapilar envolvendo a mácula. Conclusões: O comprometimento do nervo óptico foi comum em pacientes com toxoplasmose ocular. O principal tipo de comprometimento do nervo óptico foi causado pela ativação de uma lesão justapapilar antiga. O tratamento foi rapidamente eficaz, mas a acuidade visual final com melhor correção foi dependente da presença de uma cicatriz no feixe papilomacular.
Asunto(s)
Humanos , Masculino , Femenino , Niño , Adolescente , Adulto , Adulto Joven , Enfermedades del Nervio Óptico/parasitología , Enfermedades del Nervio Óptico/patología , Toxoplasmosis Ocular/patología , Nervio Óptico/patología , Nervio Óptico/diagnóstico por imagen , Retinitis/parasitología , Retinitis/patología , Factores de Tiempo , Turquía/epidemiología , Agudeza Visual , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/epidemiología , Papiledema/parasitología , Papiledema/patología , Toxoplasmosis Ocular/tratamiento farmacológico , Prevalencia , Estudios Transversales , Estudios Retrospectivos , Tomografía de Coherencia Óptica/métodos , Centros de Atención TerciariaRESUMEN
PURPOSE: To evaluate the prevalence, clinical characteristics, and types of optic nerve involvement in patients with ocular toxoplasmosis. METHODS: For this retrospective cross-sectional study, we examined all patients with active ocular toxoplasmosis referred to our Uveitis Section during the last 12 years, and we included patients with optic nerve involvement in the study. The primary outcome was the prevalence of optic nerve involvement, and secondary outcomes included the types of optic nerve involvement and the final best-corrected visual acuity after treatment. RESULTS: The prevalence of optic nerve involvement was 14.4%, with the leading cause being the activation of a juxtapapillary lesion (70.5%). We found papillitis in two eyes and neuroretinitis in two eyes (11.7% for each). We only detected one optic nerve involvement secondary to a distant active lesion (5.8%). Sixteen patients (94.1%) had unilateral ocular toxoplasmosis. The overall final best-corrected visual acuity after treatment was 10/10 (LogMAR = 0.0) excluding the three patients with a juxtapapillary scar involving the macula. CONCLUSIONS: Optic nerve involvement was common in patients with ocular toxoplasmosis. The main type of optic nerve involvement was caused by activation of an old juxtapapillary lesion. Treatment was quickly effective, but the best-corrected visual acuity was dependent on the presence of a scar in the papillomacular bundle.
Asunto(s)
Enfermedades del Nervio Óptico/patología , Enfermedades del Nervio Óptico/parasitología , Toxoplasmosis Ocular/patología , Adolescente , Adulto , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Nervio Óptico/diagnóstico por imagen , Nervio Óptico/patología , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/epidemiología , Papiledema/parasitología , Papiledema/patología , Prevalencia , Retinitis/parasitología , Retinitis/patología , Estudios Retrospectivos , Centros de Atención Terciaria , Factores de Tiempo , Tomografía de Coherencia Óptica/métodos , Toxoplasmosis Ocular/tratamiento farmacológico , Toxoplasmosis Ocular/epidemiología , Turquía/epidemiología , Agudeza Visual , Adulto JovenRESUMEN
INTRODUCTION: Functional impairment of the optic nerve is characterized by visual loss, dyschromatopsia, visual field defects, relative afferent pupillary defect, and swelling or atrophy of the optic nerve. AIM: To describe the spectrum of acute optic neuropathies, focusing on clinical features, diagnosis and treatment with an emphasis on pediatric entities. DEVELOPMENT: Optic neuritis may be monophasic, recurrent, or part of a polysymptomatic demyelinating process. The aim of the treatment is to reduce number and severity of attacks and prevent future disability. Infectious neuritis is secondary to different microorganisms (bacteria, virus, fungi, or protozoa). Treatment is related to etiology. Nonarteritic ischemic optic neuropathy or idiopathic optic neuropathy is the most frequent form and is secondary to a disorder of small retinal vessels. Leber hereditary optic neuropathy is an important cause of chronic visual impairment and is characterized by selective involvement of the retinal ganglion cells. Until now, no curative treatment is available. Visual acuity is frequently conserved in papilledema associated with intracranial hypertension. The aim of treatment is to reduce intracranial hypertension and risk factors in case it is secondary. CONCLUSIONS: Acute optic neuropathies are broad group of entities, of different etiologies, and with a variable visual prognosis. Findings of neurological examination, fundoscopy, and neuroimaging guide diagnosis and prompt treatment.
TITLE: Neuropatia optica aguda: diagnosticos diferenciales.Introduccion. La alteracion funcional del nervio optico se caracteriza por un deficit en la agudeza visual, en la vision cromatica y en el campo visual, defecto pupilar aferente y, en algunos casos, edema del nervio o atrofia y palidez. Objetivo. Describir el espectro de neuropatias opticas agudas, su clinica, diagnostico y tratamiento, con mayor interes en aquellas de presentacion en la edad pediatrica. Desarrollo. La neuritis optica puede ser monofasica, recurrente o el componente de un cuadro desmielinizante polisintomatico. El objetivo del tratamiento es reducir el numero y la gravedad de los ataques y prevenir discapacidad. La infecciosa es secundaria a diferentes microorganismos (bacterias, virus, hongos y protozoos). El tratamiento depende de la etiologia. La isquemica anterior no arteritica o idiopatica es la forma mas frecuente y es secundaria a enfermedad de pequeños vasos (ciliares posteriores). La neuropatia optica hereditaria o de Leber representa una causa importante de afectacion visual cronica y se caracteriza por la afectacion selectiva de las celulas ganglionares de la retina. Hasta el momento, la terapia solo es de apoyo. En el papiledema asociado a hipertension endocraneal, la agudeza visual generalmente se conserva pero existe aumento de la mancha ciega. El tratamiento se basa en disminuir la hipertension y el factor etiologico si existe. Conclusiones. Las neuropatias opticas agudas constituyen un amplio grupo de entidades, de etiologia diversa y con un pronostico visual variable. La presencia de signos del examen neurologico, fondo de ojo y neuroimagenes pueden orientar hacia el diagnostico y tratamiento oportuno.
Asunto(s)
Enfermedades del Nervio Óptico/diagnóstico , Enfermedad Aguda , Corticoesteroides/uso terapéutico , Antiinfecciosos/uso terapéutico , Niño , Enfermedades Desmielinizantes/complicaciones , Diagnóstico Diferencial , Técnicas de Diagnóstico Oftalmológico , Endoftalmitis/complicaciones , Endoftalmitis/tratamiento farmacológico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Hipertensión Intracraneal/complicaciones , Neuroimagen , Atrofia Óptica Hereditaria de Leber/diagnóstico , Atrofia Óptica Hereditaria de Leber/genética , Nervio Óptico/irrigación sanguínea , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/etiología , Neuritis Óptica/diagnóstico , Neuritis Óptica/tratamiento farmacológico , Neuritis Óptica/etiología , Neuropatía Óptica Isquémica/diagnóstico , Papiledema/etiología , Pronóstico , Seudotumor Cerebral/complicaciones , Retinitis/diagnóstico , Agudeza VisualRESUMEN
We described a 35 years old female patient with bilateral visual loss and pain on eye movement, mild papillary edema in acute phase, arcuate scotoma and complementary test positive for antinuclear antibodies that did not respond to corticosteroid therapy. The lack of clinical criteria for systemic lupus erythematosus (SLE) didn't prevent the institution of the specific treatment with corticosteroids and azathioprine. After seven months the diagnosis was made after a skin manifestation of the disease. This case shows the value of the ocular complaints in systemic diseases. And how the ophthalmologic exam can help the clinician elaborating a diagnosis. It is also very important for ophthalmologists and rheumatologists due to the fact that it calls the attention to another diagnostic hypothesis in patients with nonspecific optic neuritis, even with inconclusive laboratory tests. Maybe some ocular findings deserve to be included to the diagnostic criteria already established for SLE.
Descrevemos caso de um paciente de 35 anos do sexo feminino, com perda visual bilateral associada à dor à movimentação ocular, edema papilar moderado na fase aguda, escotoma arqueado e exame complementar positivo para anticorpos antinucleares, que não responderam à terapia com corticosteróides. A falta de critérios clínicos para o lúpus eritematoso sistêmico (LES) não impediu a instituição do tratamento específico com corticosteróides e azatioprina. Depois de sete meses, o diagnóstico foi feito após uma manifestação da doença de pele. Este caso mostra o valor das queixas oculares em doenças sistêmicas e como o exame oftalmológico pode ajudar o clínico na elaboração de um diagnóstico. Também é muito importante para oftalmologistas e reumatologistas, devido ao fato de que chama a atenção para outra hipótese diagnóstica em pacientes com neurite óptica não-específica, mesmo com os testes laboratoriais conclusivos. Talvez alguns achados oculares merecem ser incluídos com os critérios de diagnóstico já estabelecido para o LES.
Asunto(s)
Humanos , Femenino , Adulto , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/etiología , Enfermedades del Nervio Óptico/inmunología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Oftalmoscopía , Nervio Óptico/patología , Pregnenodionas/uso terapéutico , Piel/patología , Azatioprina/uso terapéutico , Biopsia , Proteína C-Reactiva/metabolismo , Prednisolona/uso terapéutico , Enfermedades del Nervio Óptico/tratamiento farmacológico , Papiledema/patología , Anticuerpos Antinucleares/sangre , Hidroxicloroquina/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológicoRESUMEN
Se realizó una revisión bibliográfica con el objetivo de proporcionar una actualización de las drogas que se emplean para retrasar la aparición de esclerosis múltiple en el manejo de la neuropatía óptica inflamatoria desmielinizante. El artículo presenta el origen y la justificación de la terapia esteroidea en este grupo de enfermedad, así como los mecanismos de acción y beneficios de tratamientos más modernos como los inmunomoduladores e inmunosupresores. El trabajo también introduce muchas de las drogas con efectos neuroprotectores que se encuentran en fases experimentales, cuyo uso prevendría la neurodegeneración que se produce a nivel de las células ganglionares retinianas en esta enfermedad neurológica. Las opciones terapéuticas actuales ofrecen variantes de tratamiento adicionales a pacientes con mayores probabilidades de desarrollo de esclerosis múltiple y retrasan la aparición de un segundo brote, así como las secuelas invalidantes que esta suele originar(AU)
A bibliographic review was conducted to provide an updating of drugs used to retard the appearance of multiple sclerosis in the management of the demyelinating inflammatory optical neuropathy. Present paper shows the origin and the justification of the steroid therapy in this disease, as well as the mechanisms of action and benefits of more recent treatments, e.g. the ongoing immunomodulations and immunosuppressive ones and also to introduce many drugs in experimental phase having neuroprotection effects whose use will prevent the neurodegenerative effect produced at level of the retinal ganglion cells in this neurologic disease. The current therapeutical options offer variants of additional treatment to those patients with greater possibilities to development multiple sclerosis and retarding the appearance of a second outbreak, as well as its disabling sequelae(AU)
Asunto(s)
Humanos , Masculino , Femenino , Enfermedades del Nervio Óptico/tratamiento farmacológico , Esclerosis Múltiple/etiología , Esclerosis Múltiple/prevención & control , Esteroides/uso terapéutico , Terapia Biológica/métodosRESUMEN
Se realizó una revisión bibliográfica con el objetivo de proporcionar una actualización de las drogas que se emplean para retrasar la aparición de esclerosis múltiple en el manejo de la neuropatía óptica inflamatoria desmielinizante. El artículo presenta el origen y la justificación de la terapia esteroidea en este grupo de enfermedad, así como los mecanismos de acción y beneficios de tratamientos más modernos como los inmunomoduladores e inmunosupresores. El trabajo también introduce muchas de las drogas con efectos neuroprotectores que se encuentran en fases experimentales, cuyo uso prevendría la neurodegeneración que se produce a nivel de las células ganglionares retinianas en esta enfermedad neurológica. Las opciones terapéuticas actuales ofrecen variantes de tratamiento adicionales a pacientes con mayores probabilidades de desarrollo de esclerosis múltiple y retrasan la aparición de un segundo brote, así como las secuelas invalidantes que esta suele originar
A bibliographic review was conducted to provide an updating of drugs used to retard the appearance of multiple sclerosis in the management of the demyelinating inflammatory optical neuropathy. Present paper shows the origin and the justification of the steroid therapy in this disease, as well as the mechanisms of action and benefits of more recent treatments, e.g. the ongoing immunomodulations and immunosuppressive ones and also to introduce many drugs in experimental phase having neuroprotection effects whose use will prevent the neurodegenerative effect produced at level of the retinal ganglion cells in this neurologic disease. The current therapeutical options offer variants of additional treatment to those patients with greater possibilities to development multiple sclerosis and retarding the appearance of a second outbreak, as well as its disabling sequelae
Asunto(s)
Humanos , Masculino , Femenino , Enfermedades del Nervio Óptico/tratamiento farmacológico , Esclerosis Múltiple/etiología , Esclerosis Múltiple/prevención & control , Esteroides/uso terapéutico , Terapia Biológica/métodosRESUMEN
When Cuba was hit by a neuropathy epidemic two decades ago, Dr Rosaralis Santiesteban was one of the Cuban health professionals who played a key role in its management, as reflected in a recent issue of Seminars in Ophthalmology. She was well prepared for her part: trained in medicine at the University of Havana before completing a residency in ophthalmology and eventually a doctorate in medical sciences, she has received multiple honors for her research, publishing and teaching. In 2007, she was named Distinguished Researcher by the Cuban Ministry of Science, Technology and the Environment. She has headed the Department of Neuro-ophthalmology at Cuba's Neurology and Neurosurgery Institute since 1977. Now called Cuban Epidemic Neuropathy, the 1990s epidemic that affected over 50,000 Cubans is the largest and best-documented of its kind in history. As researchers pressed to unravel the mystery of its etiology to hasten the epidemic's end, Dr Santiesteban recognized that similar outbreaks had occurred during Cuba's wars of independence in the late 1800s--described in her book, Epidemias y Endemias de neuropatía en Cuba. In the proverbial eye of the storm during the 1990s epidemic, she shares her reflections below on the context, causes, evolution and lessons learned from the challenge that put Cuba's health system to the test.
Asunto(s)
Enfermedades del Nervio Óptico/epidemiología , Enfermedades del Sistema Nervioso Periférico/epidemiología , Trastornos de la Visión/epidemiología , Cuba/epidemiología , Brotes de Enfermedades , Femenino , Humanos , Cooperación Internacional , Masculino , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/etiología , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológico , Enfermedades del Sistema Nervioso Periférico/etiología , Administración en Salud Pública , Trastornos de la Visión/etiología , Trastornos de la Visión/rehabilitaciónRESUMEN
Dysthyroid optic neuropathy is an uncommon and severe form of presentation of Graves ophtalmopathy, caused by compression and elongation of the optic nerve. Use of high dose steroids is the treatment of choice. Decompressive surgery is reserved for refractory cases. We report a 41 years old female with a dysthyroid optic neuropathy that appeared 18 years after the diagnosis of Graves disease, manifested by a marked reduction in visual acuity. Orbit CAT scan did not show compression or elongation of optic nerve. She was treated with prednisone 60 mg per day, obtaining a complete remission after 19 days of treatment. After 90 days of follow up with low doses of steroids, the patient remains asymptomatic.
Asunto(s)
Humanos , Femenino , Adulto , Enfermedades del Nervio Óptico/etiología , Oftalmopatía de Graves/complicaciones , Enfermedad de Graves/tratamiento farmacológico , Enfermedades del Nervio Óptico/tratamiento farmacológico , Prednisona/uso terapéutico , Resultado del Tratamiento , Trastornos de la Visión/etiologíaRESUMEN
PURPOSE: In this study, we aimed to evaluate the efficacy of peribulbar triamcinolone injections to treat inflammatory signs of Graves' ophthalmopathy (GO) in patients with moderate to severe GO and associated optic neuropathy (ON). METHODS: Twenty-one patients with active GO [clinical activity score (CAS) > or = 4] and systemic thyroid disease under control were enrolled in this prospective pilot study. Peribulbar triamcinolone acetonide was injected in each orbit (42 eyes), in four doses of 20 mg at 2-week intervals. Ophthalmological examination including CAS evaluation, visual field, computerized tomography (CT) scan and digital photography were performed before and after treatment. RESULTS: Twenty-one patients (11 with moderate disease, 10 with ON) were enrolled in this study and followed for at least 14 months. Initial mean CAS was 6.38 +/- 1.49, which dropped to 1.8 +/- 1.12 after 6 months of treatment (P = 0.01; mean difference of 4.57 +/- 1.56; range 1-8 score points). ON was diagnosed in 10 patients. Of these, 66% improved with peribulbar triamcinolone exclusively. A transitory increase in intraocular pressure in two patients was controlled with topic medication. CONCLUSION: Peribulbar triamcinolone injections reduce the inflammatory signs of moderate GO, as measured by the CAS, and could also be used as an alternative treatment for ON. Randomized clinical trials are needed to compare the results of triamcinolone peribulbar injections to those of other treatment modalities.
Asunto(s)
Glucocorticoides/administración & dosificación , Oftalmopatía de Graves/tratamiento farmacológico , Enfermedades del Nervio Óptico/tratamiento farmacológico , Triamcinolona Acetonida/administración & dosificación , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Oftalmopatía de Graves/diagnóstico por imagen , Humanos , Inyecciones , Presión Intraocular/efectos de los fármacos , Masculino , Persona de Mediana Edad , Enfermedades del Nervio Óptico/diagnóstico por imagen , Órbita , Proyectos Piloto , Estudios Prospectivos , Retratamiento , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Campos Visuales/efectos de los fármacosRESUMEN
INTRODUCTION: Infection with human immunodeficiency virus (HIV) is often accompanied by neurological complications. One of these includes disorders affecting the peripheral and visual nervous system, especially during the acquired immunodeficiency syndrome (AIDS) stage. DEVELOPMENT: The peripheral neuropathies associated with infection by HIV are an assorted group of disorders, which include acute or chronic inflammatory demyelinating polyneuropathy, multiple mononeuropathy and neuropathies related to the herpes zoster virus or cytomegalovirus. The most common and clinically important of the neuropathies is painful distal sensory polyneuropathy (DSP). The most severely affected cranial nerves are V and VII. The isolation of HIV from the affected nerves suggests a direct role, but an immune mechanism is also possible. Although cytomegalovirus may be associated with a variety of peripheral nerve syndromes, its clinical presentation as a primary demyelinating polyneuropathy is unusual. CONCLUSIONS: DSP and antiretroviral toxic neuropathy are the most common HIV-associated neuropathies. Both HIV infection, by itself, and the neurotoxicity of certain drugs in tritherapy contribute to the development of painful peripheral sensory neuropathy. In researching into the cause of HIV-associated neuropathy further studies are needed to determine the relative roles played by the viral infection and the activation of the immunological factors that contribute to the pathogenesis of the damage done in axons, the dorsal root ganglion and in the sensory pathways in the spinal cord.
Asunto(s)
Infecciones por VIH , Enfermedades del Nervio Óptico/fisiopatología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Fármacos Anti-VIH/efectos adversos , Fármacos Anti-VIH/uso terapéutico , Terapia Antirretroviral Altamente Activa , Nervios Craneales/patología , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/fisiopatología , VIH-1 , Humanos , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológicoRESUMEN
A 38-year-old woman from Antigua had compressive optic neuropathy of the right eye caused by orbital involvement with sinus histiocytosis. There was also nasal sinus involvement and massive cervical lymphadenopathy resulting in radiographic compression of the airway and carotid sheath. Because of the compressive optic neuropathy and threat to the airway and carotid perfusion, the patient underwent a 6-month chemotherapeutic regimen of cyclophosphamide, vincristine, and prednisone. After chemotherapy, the visual dysfunction resolved in correlation with diminution of the orbital mass, and marked regression of the cervical lymphadenopathy. This case demonstrates the potential efficacy of chemotherapy in the treatment of compressive optic neuropathy in cases of orbital sinus histiocytosis with massive lymphadenopathy (Au)
Asunto(s)
Adulto , Femenino , Informes de Casos , Humanos , Histiocitosis Sinusal/complicaciones , Síndromes de Compresión Nerviosa/tratamiento farmacológico , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades Orbitales/complicaciones , Enfermedades Linfáticas/complicaciones , Síndromes de Compresión Nerviosa/etiología , Enfermedades del Nervio Óptico/etiologíaRESUMEN
Nearly 51,000 Cubans were afflicted during an outbreak of an optic neuropathy (ON) and peripheral neuropathy (PN) between 1991 and 1993. We re-examined 14 of 20 affected individuals 16 months after an initial evaluation. The optic features were painless symmetric vision loss with poor visual acuity, color vision loss, central or cecocentral scotoma, optic disc pallor, and nerve fiber layer drop-out. The neurologic symptoms included stocking-glove sensory changes, hearing loss, leg cramps, sensory ataxia, hyperactive or absent reflexes, and complaints of memory loss. Two of 11 ON probands tested harbored Leber's hereditary optic neuropathy (LHON)-associated mitochondrial DNA mutations. All patients had received multivitamin therapy. We performed comparisons using the paired two-tailed t test. On re-examination, 12 of 14 patients demonstrated improvement. One patient remained unchanged. One woman with the nt-3460 mtDNA mutation showed a decline in vision. In patients not harboring mtDNA mutations, overall visual acuity, color vision, and peripheral neuropathy manifestations improved significantly (p < 0.001 for each manifestation). Most of the patients with Cuban ON and PN improved on multivitamin therapy. The significance of the mtDNA mutations is unclear. In the 2 LHON patients, manifestation of the disease may have been precipitated by nutritional deficiency. Patients with poor recovery or further deterioration should be evaluated for other factors, including poor vitamin therapy compliance and alternative diagnoses.
Asunto(s)
Brotes de Enfermedades , Enfermedades del Nervio Óptico/epidemiología , Enfermedades del Sistema Nervioso Periférico/epidemiología , Adulto , Estudios de Cohortes , Percepción de Color/efectos de los fármacos , Cuba , ADN Mitocondrial/genética , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mutación , Examen Neurológico , Atrofias Ópticas Hereditarias/tratamiento farmacológico , Atrofias Ópticas Hereditarias/genética , Atrofias Ópticas Hereditarias/fisiopatología , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Sistema Nervioso Periférico/complicaciones , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológico , Resultado del Tratamiento , Agudeza Visual/efectos de los fármacos , Vitaminas/uso terapéuticoRESUMEN
An epidemic outbreak of peripheral neuropathy affected Cuba in 1992-93 resulting in 50,862 cases (national cumulative incidence rate (CIR) 461.4 per 100,000). Clinical forms included retrobulbar optic neuropathy, sensory and dysautonomic peripheral neuropathy, dorsolateral myeloneuropathy, sensorineural deafness, dysphonia and dysphagia, spastic paraparesis, and mixed forms. For epidemiological purposes, cases were classified as optic forms (CIR 242.39) or peripheral forms (CIR 219.25). Increased risk was found among smokers (odds ratio (OR) 4.9), those with history of missing meals (OR 4.7) resulting in lower intake of animal protein, fat, and foods that contain B-vitamins, combined drinking and smoking (OR 3.5), weight loss (OR 2.8), excessive sugar consumption (OR 2.7) and heavy drinking (OR 2.3). Optic neuropathy was characterized by decreased vision, bilateral and symmetric central or cecocentral scotomata, and loss of color vision due to selective lesion of the maculopapillary bundles. Peripheral neuropathy was a distal axonopathy lesion affecting predominantly large myelinated axons. Deafness produced selective high frequency (4-8 kHz) hearing loss. Myelopathy lesions combined dorsal column deficits and pyramidal involvement of lower limbs with spastic bladder. Clinical features were those of Strachan syndrome and beriberi. Intensive search for neurotoxic agents, in particular organophosphorus esters, chronic cyanide, and trichloroethylene intoxication, yielded negative results. Treatment of patients with B-group vitamins and folate produced rewarding results. Most patients improved significantly and less than 0.1% of them remained with sequelae; there were no fatal cases. Supplementation of multivitamins to the entire Cuban population resulted in curbing of the epidemic. Overt malnutrition was not present, but a deficit of micronutrients, in particular thiamine, cobalamine, folate and sulfur amino acids appears to have been a primary determinant of this epidemic.
Asunto(s)
Avitaminosis/epidemiología , Brotes de Enfermedades , Enfermedades del Nervio Óptico/epidemiología , Enfermedades del Sistema Nervioso Periférico/epidemiología , Adolescente , Adulto , Anciano , Avitaminosis/tratamiento farmacológico , Beriberi/epidemiología , Estudios de Casos y Controles , Niño , Cuba/epidemiología , Sordera/epidemiología , Femenino , Ácido Fólico/uso terapéutico , Alimentos/efectos adversos , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Trastornos Nutricionales/epidemiología , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/etiología , Paraparesia Espástica Tropical/epidemiología , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológico , Enfermedades del Sistema Nervioso Periférico/etiología , Política , Trastornos de la Sensación/epidemiología , Toxinas Biológicas/análisis , Oligoelementos/deficiencia , Complejo Vitamínico B/uso terapéuticoAsunto(s)
Brotes de Enfermedades , Enfermedades del Nervio Óptico/epidemiología , Enfermedades del Sistema Nervioso Periférico/epidemiología , Adolescente , Adulto , Anciano , Consumo de Bebidas Alcohólicas , Niño , Cuba/epidemiología , Enfermedades Carenciales/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/etiología , Enfermedades del Sistema Nervioso Periférico/tratamiento farmacológico , Enfermedades del Sistema Nervioso Periférico/etiología , Fumar , Vitamina A/uso terapéutico , Complejo Vitamínico B/uso terapéuticoRESUMEN
From January 1, 1992, through January 14, 1994, the Ministry of Public Health of Cuba (MINSAP) identified 50,862 cases of a neuropathy in residents of Cuba (1993 population: 10.8 million); affected persons had onset beginning July 1, 1991. The neuropathy has included an optic form--characterized by subacute (i.e., 3-30 days) onset, decreased visual acuity, decreased color vision, and/or central or cecocentral scotomata--and a peripheral form; both forms have been characterized by weight loss and easy fatigability. This report presents a preliminary summary of an investigation by MINSAP of this epidemic.