A 68 years old man with a previous diagnostic of Hemorragic Hereditary Telangiectasia (HHT) or Osler-Weber-Rendu Syndrome has been transferred to the emergency room of Metropolitano´s Hospital of Quito because of altered mental status, fever and nuchal rigidity. Complementary studies reported images of ventriculitis and left temporal abscess.
Reportes de Casos Clínicos
Epilepsia Parcial Benigna Atípica de la Infancia: Presentación de un Caso Peculiar y Revisión de la Bibliografía.
Introduction: Atypical benign partial epilepsy of childhood is a rare form of epilepsy characterized by a combination of partial seizures, as observed in Partial Benign Epilepsy of Childhood, as well as with centre-temporal paroxysms and generalized seizures; showing furthermore, a continuos peak wake electroencephalographic pattern characteristic of NREM sleep.
Clinical Case: Patient presenting with partial seizures is admitted to the hospital with EEG findings compatible with continuos peak wave electric status of NREM sleep.
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Enfermedad de Creutzfeldt-Jakob. Presentación de un Caso Clínico y Revisión de la Literatura.
Creutzfeldt-Jakob disease is the prototypic rapidly progressive dementia. Esporadic CJD is the most common presentation. We report a 65 year-old patient with primary visual complaints, afterward psychosis with memory and higher cerebral functions complaints. After 4 months of evolution, the patient showed akinetic mutism and myoclonus. In the MRI, anormal hyperintensity was seen on T2 sequences in the subcortical and cortical regions of the occipital lobes. EEG revealed periodic generalized triphasic waves. We present this patient with the Heindenhain variant of sporadic CJD, in order to consider it when were are faced with a patient with rapidly progressive dementia (RPD). We emphasize the importance of early diagnosis for prognostic value and for minimize the risk of accidental iatrogenic transmission.
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Partial Thenar Atrophy as a Physical Manifestation of Martin Gruber Anastomosis.
Martin Gruber anastomosis is a frequent finding on electrodiagnostic examination and has three common variants. Much has been written about these variants such as the anatomic course of crossover fibers and the electrodiagnostic findings. However, little has been written on associated physical findings that might suggest such a diagnosis. In this report the physical examination findings clearly supported a diagnosis of a Type III Martin Gruber anastomosis that was initially established through electrodiagnostic testing. Awareness of this pattern on physical examination could provide an early clue to the possible presence of anomalous innervation.
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Meningitis Crónica por Angiostrongylus Cantonensis.
Two patients with chronic meningitis due to the helmint Angiostrongylus cantonensis are reported. Two years after the primary infection from a previous outbreak, both patients had pain in lumbosacral region, leg weakness, headache and sleep disturbances among other symptoms. Intrathecal synthesis of IgA, IgM and IgG was found. Nuclear magnetic resonance showed temporal atrophy in one patient and in the other one a T2 hyperdense lesion zone in right parietal region at white zone level. It is the first report of chronic disease because of this parasite in the Americas.
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Encefalomielitis Aguda Diseminada: Reporte de un Caso con Afectación Selectiva de Tallo Cerebral.
Introduction: Acute Disseminated Encephalomyelitis (ADEM) is an autoimmune demyelinating disease of the central nervous system´s white matter; it usually has a monophasic course with an idiopathic cause or following infection or immunization processes.
Objective: This article has the purpose to present a clinical case with selective involvement of brainstem. We describe a 28 year old woman who developed ADEM. Her clinical presentation, neuroradiological findings and treatment are reported.
Conclusions: ADEM is an infrequent disease which can be fatal. MRI findings confirm the diagnosis and steroid therapy appears to be the most effective treatment, although the disease may spontaneously improve.
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Apoplexy of Rathke Cleft Cyst: A Case Report and Literature Review.
Symptomatic Rathke cleft cysts (RCCs) are rare sellar and suprasellar lesions, and apoplexy is one of the most unusual presentations. Only a few cases of hemorrhagic apoplexy of RCCs have been reported and their pathogenesis is still poorly understood. In order to present a diagnostic thread to reduce misdiagnosis rate preoperatively, we reported one case of RCC apoplexy and reviewed the associated published literature. we also summarized the clinicopathological relationship on clinical symptoms, imaging features and intraoperative visualization of intracystic content.
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Hemangioblastoma Sólido de Cerebelo. Presentación de un Caso y Revisión de la Literatura.
Hemangioblastoma is an uncommon benign and highly vascular tumor that is usually observed in the brain, spinal cord and retina. It may be solid or cystic and approximately 25 to 40% of them are associated with von Hippel-Lindau syndrome. Clinical manifestations of hemangioblastoma are nonspecific and they depend on the location and growth pattern of the tumor. Despite its great vascularity, they rarely manifest themselves with hemorrhage and when they do, intraparenchymal bleeding, intramedullary hemorrhage or subarachnoid hemorrhage may be observed. The definitive diagnosis of HB is the pathologic exam. The treatment of choice for hemangioblastoma is microneurosurgery in order to make a complete resection. Preoperative endovascular embolization of feeding vessels may be useful in selected cases as the main risk of this surgery is intraoperative bleeding. We present a case of a 25 years old female diagnosed with right cerebellar hemisphere hemangioblastoma and hydrocephalus, therefore treated through various surgical stages: placing of a medium pressure valve, suboccipital craniectomy, supraselective embolization of feeder vessels and finally extirpation of the tumor mass.
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Enfermedad de Lyme. Primer caso Reportado en Ecuador.
Lyme disease is a multisystem infectious disease caused by the tick-borne spirochete Borrelia burgdorferi. CNS involvement occurs frequently. Meningitis, multiple cranial nerve palsies, neurophaties, radiculophaties, and encefalophaties are particularly common. The diagnosis should be done carefully; epidemiology, clinical features, and laboratory findings are the basis in the management of disease. In this paper we present a 12 year old Ecuadorian boy who fulfills diagnosis parameters for Lyme disease according to the American Academy of Neurology. This is the first reported case of this disease in Ecuador. It is necessary to carry out epidemiological studies to confirm the occurrence of this condition, and the look for the presence of the transmitting vector and its causal agent in our country.
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Síndrome de Kabuki
Kabuki’s mask syndrome is a rare developmental disease. Most cases are sporadic. Diagnosis is based on five criteria, including slight mental delay slight, skeletal abnormalities, dermatological alterations, slow stature, and typical fascies. We describe the first diagnostic case of Kabuki’s syndrome in our country.
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Síndrome de POEMS: Múltiples manifestaciones clínicas durante su larga evolución.
Being a multisystemic disorder, POEMS syndrome has different clinical features.and comprises multiple organs. The referred acronym: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein M and skin changes, results insufficient when this syndrome is described, because of the constellation of multiple signs and symptoms. Neurologically a chronic demyelinating peripheral neuropathy is the common clinical presentation, which is associated with an osteosclerotic myeloma in 50% of cases. Involvement of central nervous system includes papilledema and intracraneal hypertension, less frequently. We report a patient with POEMS syndrome with 11 years follow-up, who has recently developed intracraneal hypertension features. We discuss clinical findings and review literature.
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Aneurisma Gigante de la Bifurcación de la Arteria Cerebral Media: Reconstrucción con Clipaje y Microsutura
The key for the treatment of giant intracranial aneurysms is its exclusion from the circulation and restoration of the normal anatomy. This report describes the technique of the middle cerebral artery bifurcation reconstruction using microsuture aneurysmorraphy and clipping.
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Rizotomía Prepontina para el Tratamiento de la Neuralgia Trigeminal Secundaria a Infarto de Tronco Cerebral
A 62-year-old woman presented with severe pain in the distribution of the second and third branches of the left trigeminal nerve. Pain was related to an infarct located in the brainstem at the entry of the trigeminal nerve. As pain was refractary to medical therapy, we performed a selective prepontine trigeminal rhizotomy. The procedure resulted in total relief of pain with preservation of sensation in the distribution of the trigeminal nerve.
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Palabras clave: nervio trigémino,
Rizotomía Prepontina,
Keywords: prepontine trigeminal rhizotomy,
trigeminal nerve,
Estesioneuroblastoma: Presentación de un Caso y Revisión de la Literatura
A 34-year-old man presente wiith progressive anosmia, epistaxis, and intracranial hypertension related to a nasal tumor with intracranial extension. After surgical resection of the lesion, inmunohistochemical and electrical microscopy study of the biopsy material stablished the diagnosis of esthesioneuroblastoma or olfactory neuroblastoma. This is a rare embrionary tumor derived from neuroblasts of the olfactory sensorial system. That portion of the olfactory tract is located at the upper half of the nostrils, above the cribiform plate. Esthesioneuroblastoma shows a bimodal incidence with a first peak in the second decade, and a second and most important peak during the third and fifth decades of live. Individuals from both sexes are similarly affected. Pollipoid mass with epistaxis or nasal obstruction and chronic anosmia are the most common clinical manifestations of this tumor. It is invasive and frequently causes regional and distant metastasis; however, some tumors could stay without recognition until its intracranial growing through the cribiform plate causes neurologic symptoms.
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Palabras clave: estesioneuroblastoma,
neuroblastoma olfatorio,
Keywords: esthesioneuroblastoma,
olfactory neuroblastoma,
Pseudotumor cerebral: el lado maligno del espectro clínico
Introduction. The pseudotumor cerebri syndrome (PTC) is a clinical entity of unknown etiology affecting mainly young, obese women. The major morbidity factor in this condition is the possibility of vision loss, initially considered to be low, but now known to affect a significant proportion of patients. Clinical case. We describe a case of PTC in a young, obese black women who presented with headache, transient visual blurring and diplopia. Besides obesity, there where no other relevant clinical findings, namely other pathologies and history of medication use. An extensive analytical workup was negative and the CSF showed no abnormalities, aside from increased pressure. Brain MRI was also normal. The clinical course was progressively worse, consisting of rapid vision loss associated with an intracranial pressure of over 850 mmH2 O, despite treatment with diuretics (acetazolamide, furosemide and spironolactone) in therapeutic dosage. Eventually, the patient needed the rapid implantation of a lumboperitoneal shunt to prevent blindness, and went on to make a nearly full recovery. Conclusions. In all major series of PTC, loss of vision has been reported to occur in a significant percentage of patients, and cases have been described in which a catastrophic course with rapid progression to near blindness has led to the introduction of the designation of ‘malignant’ pseudotumor cerebri. We believe that such cases, although rare, need to be recognized as the malignant end of the spectrum of PTC, and that their management constitutes a neurological emergency.
