Neuralgia del Trigémino / Trigeminal neuralgia

Resumen La neuralgia del trigémino (NT) es una enfermedad cuya prevalencia es alta y corresponde a un porcentaje importante de neuralgias faciales; en donde las personas más afectadas son mayores de 50 años. Su manifestación clínica suele ser de cuadros de dolor facial severo y recurrentes, unilateral; en la distribución de una o más divisiones del nervio trigémino y no se explica con otro diagnóstico. El diagnóstico se basa en el cuadro clínico y usualmente no se encuentra déficit sensorial, sin embargo, si está presente se deben hacer neuroimágenes para descartar otras causas. En primera instancia está el manejo farmacológico. La carbamazepina se ha establecido como efectivo, llegando a producir un alivio del dolor dentro de las 24 horas. Cuando la farmacoterapia falla, se opta por la cirugía que se divide generalmente en dos: técnicas que destruyen la porción sensitiva del nervio; y la descompresión microvascular (DMV), que es la que tiene mejores resultados.

Abstract Trigeminal neuralgia is a disease whose prevalence is high and corresponds to a significant percentage of facial neuralgia; where the most affected people are over 50 years old. The clinical picture is usually of episodes of severe and recurring facial pain, unilateral; in the distribution of one or more divisions of the trigeminal nerve and this is not explained with another diagnosis. Diagnosis is based on the clinic and usually no sensory deficit is found, however, if present, neuroimaging should be done to rule out other causes. In the first instance is the pharmacological management. Carbamazepine has been established as effective, leading to pain relief within 24 hours. When pharmacological therapy fails, surgery is generally divided into two: techniques that destroy the sensitive portion of the nerve and microvascular decompression, which has the best results.

Propentofylline reverses delayed remyelination in streptozotocin-induced diabetic rats

Objective The diabetic state induced by streptozotocin injection is known to impair oligodendroglial remyelination in the rat brainstem following intracisternal injection with the gliotoxic agent ethidium bromide (EB). In such experimental model, propentofylline (PPF) recently showed to improve myelin repair, probably due to its neuroprotective, antiinflammatory and antioxidant effects. The aim of this study was to evaluate the effect of PPF administration in diabetic rats submitted to the EB-demyelinating model. Materials and methods Adult male rats, diabetic or not, received a single injection of 10 microlitres of 0.1% EB solution into the cisterna pontis. For induction of diabetes mellitus the streptozotocin-diabetogenic model was used (50 mg/kg, intraperitoneal route – IP). Some diabetic rats were treated with PPF (12.5 mg/kg/day, IP route) during the experimental period. The animals were anesthetized and perfused from 7 to 31 days after EB injection and brainstem sections were collected for analysis of the lesions by light and transmission electron microscopy. Results Diabetic rats injected with EB showed larger amounts of myelin-derived membranes in the central areas of the lesions and considerable delay in the remyelinating process played by surviving oligodendrocytes and invading Schwann cells after the 15th day. On the other hand, diabetic rats that received PPF presented lesions similar to those of non-diabetic animals, with rapid remyelination at the edges of the lesion site and fast clearance of myelin debris from the central area. Conclusion The administration of PPF apparently reversed the impairment in remyelination induced by the diabetic state. Arch Endocrinol Metab. 2015;59(1):47-53 .

Dermatofibrosarcoma protuberans / Dermatofibrosarcoma protuberans

El dermatofibrosarcoma protuberans es un sarcoma de partes blandas, de malignidad intermedia y lento crecimiento. Afecta adultos jóvenes y a todas las etnias, aunque algunos trabajos observan cierta predilección por la etnia negra. La localizaciónhabitual es en tronco, extremidades proximales y en menor medida encabeza y cuello. El tratamiento de elección es la exéresis quirúrgica con preferencia por la cirugía micrográfica de Mohs. Presentamos seis pacientes con diagnóstico clínico e histopatológico de dermatofibrosarcoma protuberans, 2 mujeres y 4 varones de entre 24 a 52 años. Hasta el momento, 5 de ellos recibieron tratamientoquirúrgico (técnica micrográfica de Mohs en 4 pacientes y cirugía convencional en el restante). No se observaron recidivas en un período de seguimiento promediode 20 meses...

Brain Stem Hypoplasia Associated with Cri-du-Chat Syndrome

Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

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.

Synergistic convergence and split pons in horizontal gaze palsy and progressive scoliosis in two sisters.

Synergistic convergence is an ocular motor anomaly where on attempted abduction or on attempted horizontal gaze, both the eyes converge. It has been related to peripheral causes such as congenital fibrosis of extraocular muscles (CFEOM), congenital cranial dysinnervation syndrome, ocular misinnervation or rarely central causes like horizontal gaze palsy with progressive scoliosis, brain stem dysplasia. We hereby report the occurrence of synergistic convergence in two sisters. Both of them also had kyphoscoliosis. Magnetic resonance imaging (MRI) brain and spine in both the patients showed signs of brain stem dysplasia (split pons sign) differing in degree (younger sister had more marked changes).

Pontine haemorrhage due to chondrosarcoma of the skull base

young adult presented with acute weakness of right side of body and slurring of speech. An initial brain CT scan showed a pontine haemorrhage, however MRI done a few days later revealed a mass in the left parapharyngeal space. Histopathology of the mass revealed that it was a chondrosarcoma. Subsequently the patient was initially treated with chemotherapy and radiotherapy and later surgery. Skull base chondrosarcomabe is to be included in the differentials of a young patient presenting with signs consistent with pontine haemorrhage

Presumed Metastasis of Breast Cancer to the Abducens Nucleus Presenting as Gaze Palsy

A 51-year-old woman with breast cancer presented with progressive diplopia. Neuro-ophthalmologic examination revealed right gaze palsy and peripheral facial nerve palsy. Brain magnetic resonance imaging (MRI) was normal. However, two months later a repeat brain MRI revealed an enhancing round nodular mass at the right facial colliculus of the lower pons, at the location of the abducens nucleus. Localized metastasis to the abducens nucleus can cause gaze palsy in a patient with breast cancer.

Further diffusion tensor imaging contribution in horizontal gaze palsy and progressive scoliosis / Contribuição adicional das imagens por tensores de difusão em paralisia do olhar conjugado horizontal associada a escoliose progressiva

In two siblings with clinical diagnosis of horizontal gaze palsy associated with progressive scoliosis (HGPPS) we could demonstrate by diffusion tensor imaging: (1) An anterior displacement of the transverse pontine fibers; (2) Posterior clumping of the corticospinal, medial lemniscus and central tegmental tracts and of the medial and dorsal longitudinal fasciculi complex; (3) Absent decussation of superior cerebellar peduncle. Those findings can contribute as surrogate markers for the diagnosis.

Em dois irmãos com diagnóstico clínico de paralisia do olhar conjugado horizontal associada a escoliose progressiva, foi possível determinar através de imagens por tensores de difusão: (1) Deslocamento anterior das fibras pontinas transversas; (2) Agrupamento posterior do trato córtico-espinhal, lemnisco medial e trato tegmentar central e complexos dos fascículos longitudinais medial e dorsal; (3) Ausência da decussação dos pedúnculos cerebelares superiores. Tais achados podem contribuir como marcadores para o diagnóstico.

Massive pontine hemorrhagic transformation associated with an anticoagulant for basilar artery occlusion.

Symptomatic hemorrhagic transformation is common in supratentorial and cerebellar infarction, but is rare in brainstem infarction. It is seldom reported in basilar artery occlusion. Although early arterial recanalization by thrombolytic agent has became the new trend of treatment, for some neurologists anticoagulant is still a conventional alternative treatment of basilar artery occlusion, especially in longer-existing ischemic deficits. We report a case of massive pontine hemorrhage associated with enoxaparin (low-molecular-weight heparin) treatment for basilar artery occlusion. On the basis of the clinical information and neuroimaging, an embolism was the most likely cause of stroke. The case presented herein adds massive pontine hemorrhagic transformation to the list of possible complications of anticoagulants for basilar artery occlusion. Apart from no evidence-based benefit in treatment of basilar artery occlusion, anticoagulant may contribute to devastating hemorrhagic transformation.

Parkinsonism and recovery in central and extrapontine myelinolysis.

Central pontine myelinolysis is a demyelinating affection of central pons diagnosed on the basis of characteristic MRI finding in an appropriate clinical setting. The condition has been described as universally fatal; however, recent reports of recovery have been documented. We report a case of central pontine and extra pontine myelinolysis, which presented with parkinsonian features apart from bulbar symptoms and made a remarkable recovery. A short review of the literature follows.

Internuclear ophthalmoplegia and torsional nystagmus: an MRI correlate.

The association of internuclear ophthalmoplegia (INO) with torsional nystagmus is rare. We report a case of a 72-year-old male who developed brainstem stroke and was found to have left INO with torsional nystagmus. An MRI correlation in this case has been described.

Wasp sting induced neurological manifestations.

Wasp stings generally cause local reactions like pain, wheal, flare, edema and swelling, which are generally self-limiting. Multiple stings can lead to vomiting, diarrhea, generalized edema, dyspnea, hypotension, collapse, renal failure or death. Unusually, they may cause serum sickness, vasculitis, neuritis or encephalitis. We report a case of a 40 year old male who developed focal neurological deficit 10 hours following a wasp sting, which was confirmed to be ponto-cerebellar infarction on MRI scan, and recovered within five days.

Acute disseminated encephalomyelitis--another cause of post malaria cerebellar ataxia.

The aetio-pathogenesis of delayed onset cerebellar ataxia following Plasmodium falciparum malaria is uncertain. An autoimmune demyelinating pathology has been suspected though not yet definitively substantiated. In the present communication we report a case of delayed onset cerebellar ataxia following acute falciparum malaria, where magnetic resonance imaging revealed demyelinating lesions in the pons and cerebellar peduncles which disappeared after resolution of symptoms.

Incontinência do choro e infarto protuberancial unilateral / Crying incontinence and unilateral pontine infarction

O presente estudo trata do caso de um paciente que apresentou incontinência do choro e hemiplegia direita por infarto ventroprotuberancial paramediano detectado pela RNM. O caráter circunscrito da lesäo foi endossado pela normalidade dos potenciais evocados sômato-sensitivos e auditivos de curta-latência. Os episódios de choro desapareceram poucos dias depois do início do tratamento com doses baixas de imipramina. Discutimos o choro e o riso patológicos como forma de incontinência da mímica resultante de desconexäo límbico-motora enfatizando a impropriedade de incluí-los na síndrome pseudobulbar, uma vez que dependem de correlatos anatômicos e funcionais distintos