ABSTRACT
Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group ofgenetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are consideredmajor criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.
Subject(s)
Humans , Male , Adult , Hamartoma Syndrome, Multiple/surgery , Hamartoma Syndrome, Multiple/physiopathology , Hamartoma Syndrome, Multiple/diagnostic imaging , Ganglioneuroma/physiopathology , Cerebellar Neoplasms , Cerebellum/abnormalities , Craniotomy/methodsABSTRACT
Context: Chronic subdural hematomas are frequent diseases in neurosurgery units. Decreased level of consciousness is observed in 28 to 100 percent of cases, nevertheless in rare cases are seen comatous patients. We describe a case of a patient with clinical uncal herniation elapsed by a chronic subdural hematoma. Case report: Patient with previous diagnosis of breast cancer, presented sudden occurrence of a headache followed by decreasing level of consiousness the day before reaching our service. The patient was taken than to another Hospital at 1:00 AM in Glasgow Coma Scale (GCS) 13, with isochonic pupils. At 7:00 AM the GCS went to 7 and the pupils became anisochonic L > R and than transferred to our Unit and immediatly submitted to a computed tomohraphy scanning of the head that showed a chronic subdural hematoma with midline shift. The patient promptly was taken to surgical room and treated with a single burr hole with drainage of hypertensive chronic subdural hematoma. The level of consiousness increased few hours after surgery, being in GCS 15 just 6 hours after, without motor deficits. Conclusion: In spite of commonly progressive evolution chronic subdural hematomas can present with herniation, becoming itself a neurosurgical emergency.