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.
Reportes de Casos Clínicos
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.
