Voz hipernasal y regurgitación nasal aguda: reporte de un caso clínico y revisión de la literatura / Hypernasal speech and acute nasal regurgitation: a case report and a review of the literature

RESUMEN La voz hipernasal y la regurgitación nasal son síntomas de disfunción velofaríngea. Ésta puede tener múltiples causas: anatómicas, neurológicas o funcionales. Se describe el caso de una paciente de sexo femenino, de 13 años, que se presenta con voz hipernasal y regurgitación nasal aguda. Al examen físico se evidencia inmovilidad del velo del paladar derecho sin otros hallazgos neurológicos. El estudio con resonancia nuclear magnética de cerebro y punción lumbar fueron normales. Se diagnosticó una incompetencia velofaríngea aguda transitoria, de probable etiología viral. La paciente evolucionó de forma favorable con mejoría clínica progresiva. La incompetencia velofaríngea a causa de una paresia o parálisis del nervio vago y/o nervio glosofaríngeo es una causa poco frecuente de disfunción velofaríngea.

ABSTRACT Hypernasal speech and nasal regurgitation are symptoms of velopharyngeal dysfunction. This may have multiple causes, including velopharyngeal incompetence due to paresis or paralysis of the vagus nerve and/or glossopharyngeal nerve. We describe the case of a 13 year-old female patient, with hypernasal speech and acute nasal regurgitation, with a physical examination showing immobility of the right palate with no other neurological findings. Magnetic resonance imaging of the brain and lumbar puncture was normal. Transient acute velopharyngeal incompetence was diagnosed, probably of viral etiology. The patient evolved favorably with progressive clinical improvement. Velopharyngeal incompetence due to paresis or paralysis of the vagus and/or glossopharyngeal nerves is a rare cause of velopharyngeal dysfunction.

Acometimento de nervos cranianos na granulomatose com poliangeíte (GPA) C-ANCA negativo / Cranial nerve impairment in granulomatosis with polyangeitis (GPA) C-ANCA negative

RESUMO O presente relato tem o objetivo de mostrar um caso incomum de Granulomatose com Poliangeíte (GPA), que previamente era denominada Granulomatose de Wegener. Trata-se de é uma doença multissistêmica, caracterizada por inflamação granulomatosa necrotizante e vasculite que envolve principalmente o trato respiratório superior e inferior, embora não raramente, exista comprometimento neurológico.

ABSTRACT This report aims to show an unusual case of granulomatosis with polyangeitis (GPA), previously known as Wegener's granulomatosis. It is a multisystemic disease characterized by necrotizing granulomatous inflammation and vasculitis involving mainly the upper and lower respiratory tract, although not infrequently, there is neurological impairment.

Síndrome de Villaret y adenocarcinoma de pulmón: una asociación infrecuente / Villaret syndrome and lung adenocarcinoma: an unusual association

El síndrome de Villaret se define por la afección de los nervios craneales glosofaríngeo (IX), vago (X), espinal (XI) e hipogloso mayor (XII), en conjunción con el síndrome de Horner homolateral a la lesión. Se produce por compresión de estos nervios y de las fibras vecinas del plexo simpático pericarotídeo en la base del cráneo, en particular, en el espacio retroparotídeo. Si bien es un hecho conocido la invasión del sistema nervioso central en el cáncer de pulmón avanzado, esta particular asociación sintomática es extremadamente infrecuente. Presentamos una paciente con diagnóstico reciente de adenocarcinoma de pulmón que desarrolló, en forma simultánea, este síndrome.

Villaret syndrome is defined by the affection of the glossopharyngeal (IX), vagal (X), accessory (XI) and hypoglossal (XII) cranial nerves associated with ipsilateral Horner syndrome. It is caused by the compression of these nerves and the neighboring sympathetic plexus fibers at the base of the skull, particularly in the retroparotid space. Even though the invasion of the central nervous system in patients with advanced lung cancer is a frequent and well known occurrence, this particular symptomatic association is extremely rare. We are reporting the case of a newly diagnosed lung adenocarcinoma patient who is simultaneously developing this syndrome.

Síndrome de Collet-Sicard como forma de presentación de fracturas combinadas del atlas y cóndilos occipitales / Collet-Sicard syndrome as a presentation of combined fractures of atlas and occipital condyles

El síndrome de Collet - Sicard consiste en una afectación unilateral y combinada de los nervios craneales bajos, originado por lesiones en la base craneal. CASO CLÍNICO: Paciente masculino con antecedentes de sufrir accidente del tránsito. A su llegada al hospital refiere dolor cervical alto, voz apagada y dificultad para tragar. Al examen físico se contacta paresia de pares craneales bajos. Se realizan Rx de columna cervical y tomografía axial de región cráneo espinal. Se diagnostica una fractura de los cóndilos occipitales y una fractura tipo II del atlas. Se coloca un Halo chaleco. Se consulta al paciente al final del primer mes de tratamiento con alivio del dolor cervical y sin empeoramiento neurológico. CONCLUSIONES: La afectación de pares craneales bajos puede ser la forma clínica de presentación de las lesiones traumáticas de la región cráneo espinal. Su reconocimiento temprano favorece el pronóstico de estos pacientes

Collet syndrome - Sicard is a combined unilateral involvement and lower cranial nerves, caused by damage to the cranial base. Case report. Male patient with a history of developing traffic accident. Upon arrival at the hospital referred high cervical pain, muffled voice and difficulty swallowing. Physical examination contact lower cranial nerve paresis. Rx are performed CT cervical spine and skull spinal region. Was diagnosed with a fracture of the occipital condyles and type II fracture of the atlas. Place a Halo vest. They see patients at the end of the first month of treatment with cervical pain relief without neurological deterioration. CONCLUSIONS: The lower cranial nerve involvement may be the clinical presentation of traumatic injuries of the skull spinal region. Its early recognition and the prognosis of these patients.

Brainstem cavernomas: a surgical challenge / Cavernomas de tronco: desafio cirúrgico

Objective: The authors show their experience with brainstem cavernomas, comparing their data with the ones of a literature review. Methods: From 1998 to 2009, 13 patients harboring brainstem cavernomas underwent surgical resection. All plain films, medical records and images were reviewed in order to sample the most important data regarding epidemiology, clinical picture, radiological findings and surgical outcomes, as well as main complications. Results: The mean age was 42.4 years (ranging from 19 to 70). No predominant gender: male-to-female ratio, 6:7. Pontine cases were more frequent. Magnetic resonance imaging was used as the imaging method to diagnose cavernomas in all cases. The mean follow-up was 71.3 months (range of 1 to 138 months). Clinical presentation was a single cranial nerve deficit, VIII paresis, tinnitus and hearing loss (69.2%). All 13 patients underwent resection of the symptomatic brainstem cavernoma. Complete removal was accomplished in 11 patients. Morbidity and mortality were 15.3 and 7.6%, respectively. Conclusions: Cavernomas can be resected safely with optimal surgical approach (feasible entry zone) and microsurgical techniques, and the goal is to remove all lesions with no cranial nerves impairment.

Objetivo: Os autores mostram sua experiência com cavernomas de tronco cerebral, comparando seus dados com os de uma revisão da literatura. Métodos: De 1998 a 2009, 13 pacientes com cavernoma de tronco cerebral foram submetidos a ressecção cirúrgica. Todos os filmes, prontuários e imagens foram revisados para exposição dos dados mais importantes, como epidemiologia, detalhes clínicos, achados radiológicos e resultados cirúrgicos, bem como as principais complicações. Resultados: A média de idade foi de 42,4 anos (variação de 19 a 70). Não houve predominância de gênero na taxa masculino versus feminino, 6:7. Os casos pontinos foram os mais frequentes. Ressonância nuclear magnética foi o método de imagem para o diagnóstico de cavernomas em todos os casos. A média do acompanhamento foi de 71,3 meses (variação de 1 a 138 meses). A apresentação clínica mais frequente foi a paresia do VIII nervo craniano, tinitus e perda auditiva (69,2%). Todos os 13 pacientes com cavernomas de tronco sintomáticos foram submetidos à ressecção cirúrgica. A remoção total foi realizada em 11 pacientes. A morbidade e a mortalidade foram de 15,3 e 7,6%, respectivamente. Conclusão: Os cavernomas podem ser seguramente ressecados por meio de acessos cirúrgicos ideais (zonas de entrada seguras) e técnicas de microcirurgia, sendo que o objetivo é remover toda a lesão sem o comprometimento dos nervos cranianos.

Revisión bibliográfica del uso de láser de baja potencia como tratamiento en alteraciones del nervio alveolar inferior en osteotomía sagital de rama / Literature review of the use of low power laser therapy for treating inferior alveolar nerve disorder in sagittal ramus osteotomy

Una de las principales complicaciones al realizar la osteotomía sagital de rama, es la presencia de alteraciones sensitivas que se producen a nivel del nervio alveolar inferior. La parestesia luego de esta técnica reporta una incidencia entre el 85-87 por ciento, de los cuales entre el 0-24 por ciento queda con parestesia permanente. Dentro de los tratamientos utilizados para recuperar las alteraciones sensitivas se encuentra el uso de láser de baja frecuencia; el cual se utiliza para acelerar la recuperación sensitiva y la reparación neuronal, disminuir el dolor y restaurar el funcionamiento normal del nervio injuriado. Los objetivos de esta investigación fueron realizar una revisión bibliográfica sobre los resultados obtenidos en pacientes sometidos a osteotomía sagital de rama que presentaron alteraciones sensitivas y en cuyo tratamiento se utilizó el láser, y crear un protocolo de tratamiento con láser en pacientes sometidos a osteotomía sagital de rama madibular. Se realizó una revisión de artículos entre los años 1990-2010, utilizando buscadores como: EBSCO, Cochcrane, TripDataBase, Medline, Lilacs, Pubmed y Decs, además de una búsqueda en revistas científicas. Los resultados de los artículos seleccionados señalan el efecto beneficioso que conlleva el uso del láser, demostrando su eficacia en la recuperación precoz de la sensibilidad postoperatoria. El uso del láser de baja frecuencia es una alternativa favorable para pacientes con alteraciones sensitivas que fueron sometidos a cirugía sagital de rama, debido a su pronta, mejor y mayor recuperación de la sensibilidad...

A major complication that occurs at the sagittal ramus osteotomy, is the presence of damage occurring at the inferior alveolar nerve. An incidence of paresthesia after this technique reported at 85-87 percent, of which between 0-24 percent are left with permanent paresthesia. Among the treatments used to restore sensitivity is the use of low level laser therapy, which is used to speed recovery and repair sensitive neurons, reduce pain, and restore normal functioning to injured nerve. The aims were to undertake a literature review of empiric research on the results obtained in patients under going sagittal ramus osteotomy, who has sensory disturbances and in wich laser treatment was used, and create a laser treatment protocol in patients undergoing sagittal ramus osteotomy. A review of articles between the years 1990-2010, using search engines such as EBSCO, Cochcrane, Tripdatabase, Medline, Lilacs, Pubmed and Decs. The review also included a search in scientific journals. The results of the reviewed literature showed the beneficial effect of the use of laser, demonstrating its effectiveness in the early recovery of postoperative sensitivity. The use of low level laser therapy is a favorable alternative for patients with sensory impairment who underwent sagittal ramus osteotomy due to its rapid and greater sensitivity recovery...

Disfunción de nervios craneales: tratamiento quirúrgico / Dysfunction of cranial nerve: surgical treatment

Se describen los Síndromes de disfunción de nervios craneales V- VII-VIII-IX-X y XII y la evolución histórica que ha tenido su tratamiento quirúrgico. Se discute la posible etiología, fundamento de la terapia quirúrgica. Se presenta la principal experiencia internacional, la del autor y los resultados. Se enfatiza la indicación en afecciones de tratamiento neuro quirúrgico poco común como el tortícolis espasmódico y ciertos casos de hipertensión arterial esencial. Se destaca la necesidad de alto nivel en técnica microquirúrgica.

A description and historical review of the craneal nerves disfunctions surgically treated, the possible etiopathogenesis ís discussed and the principal international and author experience is presented.

Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico / Neurological involvement in Wegener's granulomatosis: Report of one case

Wegener's granulomatosis is a systemic necrotizing vasculitis that affects medium size and small vessels. Neurological involvement occurs in 22 percent to 54 percent of patients, mainly in the form of mononeuritis multiplex. Central nervous system involvement is reported in only 2 percent to 8 percent of the cases. We report a 42-year-old male who presented with headache, diplopia, third and sixth cranial nerve palsies and íeft eye amaurosis associated to mass located in the íeft Meckel cavum and diffuse meningeal involvement. A biopsy of the mass disclosed a chronic granulomatous necrotizing inflammation with Langhans giant cells. A chest CAT scan showed three cavitated lung nodules and ANCA antibodies were positive in a titer of 1:80. Treatment with steroid and cyclophosphamide was started and cranial nerve palsies resolved and the number and size of lung nodules decreased. The patient was lost from follow up.

Acute disseminated encephalomyelitis in childhood; epidemiologic, clinical, and laboratory features

Acute disseminated encephalomyelitis [ADEM], which occurs spontaneously or after systemic viral infection and vaccination mostly affects children. This study aims at describing the epidemiologic, clinical, neuroimaging, laboratory features, treatment and outcome in children who diagnosed as having ADEM and admitted in a referral center in Tehran. This descriptive prospective study was conducted on patients with a diagnosis of ADEM over a period of 30 months, between Sep 2003 and Mar 2006, admitted to the neurology ward of Mofid Pediatric Hospital. All these patients were visited in regular follow-up every 6 months for two years. Eighteen patients with the mean age of 6 years [range 5 months - 12 years] were studied. No sex predominance was noted. Sixty-one percent had prodromal illness, and two patients had been vaccinated before the illness. Thirteen [72%] patients presented in fall or winter. Motor deficits and cranial nerve palsy were the most common features, which had occurred in 13 [72%] patients. Abnormal findings in cerebrospinal fluid evaluation were detected in 33% of the patients. Brain computed tomography was normal in all but one patient. Electroencephalograms done in nine patients were normal in 54%. Magnetic resonance imaging showed lesions were most commonly in the subcortical and periventricular areas [76%]. Nine [50%] patients were treated with corticosteroids and intravenous immunoglobulin. The mortality rate was 5.5%, and the relapse occurred only in one case. In two-thirds of the patients, prognosis for complete recovery was excellent. Childhood ADEM is a benign condition, affecting both sexes equally. Recurrent infections have been its common cause in our center

Dissecção espontânea de artéria carótida interna com paralisia de nervos cranianos inferiores: relato de caso / Spontaneous internal carotid artery dissection with paralysis of lower cranial nerves: case report

A dissecção espontânea da artéria carótida interna é evento raro, acometendo principalmente indivíduos em idade produtiva. Como a abordagem precoce e adequada dessa doença determina quase sempre melhora significativa, torna-se evidente a necessidade de ampliar os conhecimentos sobre seus aspectos clínicos, patogênicos e fisiopatológicos, visando o diagnóstico e tratamento precoces, bem como a redução de suas possíveis seqüelas. Pelos motivos expostos, julgamos pertinente relatar o caso de um homem de 46 anos com dissecção espontânea de artéria carótida interna, com manifestações clínicas atípicas dentre as quais ressaltamos a paralisia de nervos cranianos inferiores.

Spontaneous internal carotid artery dissection is a rare event that strikes patients at their productive age. As early intervention frequently determines a significant improvement, it is important to broaden the knowledge of the clinical, pathogenic and pathophysiological aspects of this disease. The objective is making an early diagnosis and treatment to avoid a bad outcome. We report the case of a 46 years-old man with spontaneous carotid artery dissection that had an atypical manifestation characterized by sudden paralysis of lower cranial nerves.

Cranial nerve involvement in patients with leprous neuropathy.

BACKGROUND: Leprosy is one of the most common causes of peripheral neuropathy, perhaps closely matched by diabetic neuropathy. Patterns of peripheral neuropathy in leprosy can be varied, which may include mononeuropathy, mononeuritis multiplex and symmetric polyneuropathy. Cranial nerves, especially facial and trigeminal nerves, are also commonly involved in leprosy. AIMS: To find out the pattern and spectrum of cranial nerve involvement in a consecutive series of patients with leprous neuropathy. SETTINGS AND DESIGN: A retrospective review of patients admitted with leprosy to the Neurology Department of a tertiary care center. MATERIALS AND METHODS: All consecutive patients admitted during an 8-year period (1995-2003) and diagnosed to have leprosy were included. They were clinically evaluated to determine the frequency and pattern of cranial nerve involvement. RESULTS: About 18% (9/51) of the leprosy patients seen during that period had clinical evidence of cranial nerve involvement. Facial and trigeminal nerves were the most commonly affected (five and four patients respectively). CONCLUSIONS: Cranial nerve involvement is common in leprosy, which emphasizes the need to carefully examine them. Also, one should exclude leprosy in patients presenting with isolated cranial neuropathies.

Mucormicosis. Reporte de tres casos / Mucormycosis. Report of 3 cases

Mucormycosis is an acute and often fatal infection caused by a fungus of the Mucorales order of the Zygomycetes class. There are various clinical types, usually associated with an underlying disorder. The rhinocerebral mucormycosis is usually seen in diabetics, especially in ketoacidosis, but may also appear in healthy people. We report three cases of mucormycosis diagnosed since april 1987 through january 2001 at the Dr. Domingo Luciani Hospital, Caracas, Venezuela. Two of them had diabetes and one was apparently healthy. The most common clinical presentation of mucormycosis was the rhinocerebral infection, seen in the two diabetic patients, both of them had cavernous sinus thrombosis one with involvement of the carotid artery. The other patient with sinus involvement had no predisposing factors. All patients were treated with amphotericin B and two of them had surgical debridement of involved tissue. We emphasize the importance of an early clinical diagnosis and treatment with surgical debridement of infected tissue combined with amphotericin B.

Hypertrophic pachymeningitis: varied manifestations of a single disease entity.

Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower cranial nerves were chiefly involved in two patients whereas optic nerve was the predominantly affected nerve in one. Except for the presence of rheumatoid arthritis in one of the patients, we could not document clinical or biochemical evidence of any predisposing infective, inflammatory or infiltrative condition in the other two. All three patients had characteristic changes on imaging suggestive of thickened and enhancing duramater. Although variable steroid responsiveness was seen in all the three patients, tendency towards steroid dependence was evident. The clinical presentations, causes, radiological features, management options and differential diagnosis of this unique clinical syndrome have been discussed.

Clinical profile of headache and cranial neuralgias.

OBJECTIVES: The present study, first of its kind from Kashmir Valley, was conducted on 2982 patients to depict the pattern of various headache types and cranial neuralgias. Besides demographic parameters, various factors influencing the frequency of headaches and cranial neuralgias were also analysed. METHODS: Patients presenting with the chief complaint of headache and facial pain were included in this study. The diagnosis of different headache types and cranial neuralgias were established after following the criteria as devised by the International Headache Society (1988). RESULTS: Mean age at presentation for all headache types/cranial neuralgias was 24.5 years (range 7-74 years) with a male:female ratio of 2:1. Tension headache and migraine was found in 1988 (66.6%) and 407 (13.6%) cases respectively. Cranial neuralgias were observed in 20 (1%) cases. CONCLUSIONS: Tension headache and migraine were the commonest forms of headache disorders. Militancy related stress and handicrafts profession were the main predisposing factors for tension and cervicogenic headaches respectively. Ramadan fasting was the prime precipitating factor for migraine.

Isolated bilateral abducens nerve palsy due to carotid cavernous dural arteriovenous fistula

Carotid cavernous dural arteriovenous fistula (DAVF) usually presents with conjunctival injection, proptosis, loss of visual acuity and ophthalmoplegia. There have been some carotid cavernous DAVF case reports presenting with isolated oculomotor, abducens and trochlear nerve palsy. We experienced a patient presenting with bilateral abducens nerve palsy and no other ocular signs who was diagnosed as carotid cavernous DAVF after conventional angiography. According to this case, carotid cavernous DAVF should be considered in the differential diagnosis of isolated bilateral abducens nerve palsy, in which case conventional angiography may be helpful in diagnosis.