Aicardi-Goutières Syndrome: Brief Case Report.

The case of a term newborn diagnosed with Aicardi-Goutières syndrome, a rare encephalopathy in our environment, with Mendelian inheritance pattern, characterized by a set of nonspecific neurological symptoms associated with typical findings of intracerebral calcifications. The case is presented with diagnostic imaging, in addition to elevated levels of interferon alpha and cerebrospinal fluid lymphocytosis.

Intradiploic Pterional Epidermoid Tumor: A Case Report and Review of literature.

Intradiploic epidermoid cyst is an uncommonly occurring neoplasm, and only about 200 cases are reported in the form of isolated case report. It is presumed to occur due to ectodermal cells in inclusion in the bone tissue during embryonic life neural tube closure. It commonly remains asymptomatic or rarely presents as a bony lump in the skull bone. Authors report present an interesting case, which presented with swelling, and underwent successful surgical resection. Pertinent literature along with diagnosis and management is briefly reviewed. These lesions can erode the bone and involve the brain parenchyma due to their proximity to the brain. Radiological imaging is very helpful in accurate diagnosis of these lesions and in differentiating intradural from intradiploic varieties of epidermoid. We present an unusual case of this pathology.

A Giant Occipital Encephalocele in Neonate with Spontaneous Hemorrhage into the Encephalocele Sac: Surgical Management.

The presence of giant occipital encephalocele represents a surgical challenge. However, preoperative magnetic resonance imaging with venography can help in delineating relation of venous sinus, content of the sac and help classify occipital encephalocele into infra-torcular and torcular depending on the relation with position of torcula. However, the presence of old hemorrhage into encephalocele sac is extremely rare and in the detailed PubMed search, the authors could find one such case, reported by Nath et al. The author reports a case of giant occipital encephalocele; during surgery, evidence of old bleed was noted. Pertinent literature and management are reviewed briefly.

[Type I Chiari malformation associated with cerebellar atrophy. Case report]. / Malformación de Chiari Tipo I asociado a atrofia cerebelosa. Reporte de caso.

Chiari malformation is characterized by caudal displacement of the cerebellar tonsils that penetrate into the spinal canal through the foramen magnum, achieving reach the atlas or axis. trunk and any drop of the fourth ventricle is observed. Typically is seen in young adults. In some cases scoliosis and Syringomyelic cavities may occur. The authors present (as far as they know) the first case in the literature with long term follow-up, of a caucasian woman with an unusual form of cerebellar atrophy and Chiari Type I malformation, suffering from weakness in his upper and lower extremities with rapidly progression. The patient was successfully treated with suboccipital decompression and C1 laminectomy.

La malformación de Chiari se caracteriza por un desplazamiento caudal de las amígdalas cerebelosas que penetran hacia el canal raquídeo por el foramen mágnum, logrando llegar hasta el atlas o el axis. No hay descenso del tronco y tampoco del cuarto ventrículo. Típicamente se observa en adultos jóvenes. En algunos casos hay escoliosis y cavidades siringomiélicas. Los autores presentan lo que a su conocimiento es el primer caso de la literatura con seguimiento a largo plazo de una mujer de raza blanca con una forma inusual de atrofia cerebelosa y malformación de Chiari tipo I, que sufre de debilidad en sus extremidades superiores e inferiores rápida y progresivamente. La paciente fue tratada con éxito mediante descompresión suboccipital y laminectomia C1.

Mycotic Cerebral Aneurysm in a Premature Infant.

Mycotic cerebral aneurysms in neonates are extremely rare and can be difficult to diagnose without a high index of suspicion. We describe a 33-week gestation preterm neonate who developed a mycotic cerebral aneurysm leading to death before repair could be performed. We believe this to be the first literature reported case of a mycotic cerebral aneurysm in a preterm infant. This case revives the ongoing question of when to perform a lumbar puncture in a potentially septic or meningitic infant, while also casting light on maintaining a high index of suspicion for rare intracranial diagnoses that require cranial imaging.

Intra-fourth Ventricular Schwannoma in Pediatric Age Group: Report of Second Case in the Western Literature with Review of Literature.

Schwannoma originates from the myelin sheath of peripheral nerves. It accounts for about 8% of all intracranial tumors. Commonly, schwannoma is located in the extra-axial locations; intra-axial schwannomas are extremely uncommon. The schwannoma arising from fourth ventricle is extremely uncommon and authors in a detailed PubMed and MEDLINE search could find only seven cases reported in the literature in the form of isolated cases report, who were managed surgically, with only one being a pediatric case. Authors report second case of intra-fourth ventricular schwannoma occurring in the pediatric age group. The hypothesis of intraventricular schwannoma is postulated to be aberrantly placed multipotent cell during embryogenesis and later transforming into Schwann cell and producing schwannoma. Pertinent literature is reviewed along with diagnosis, and management of such rare case is discussed briefly.

Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report.

Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition.

Bilateral Traumatic Basal Ganglia Hemorrhage Associated With Epidural Hematoma: Case Report and Literature Review.

Traumatic basal ganglia hematoma is a rare condition defined as presence of hemorrhagic lesions in basal ganglia or adjacent structures suchas internal capsule, putamen and thalamus. Bilateral basal ganglia hematoma are among the devastating and rare condition. We herein report a 28-year old man, a victim of car-car accident who was brought to our surgical emergency room by immediate loss of consciousness and was diagnosed to have hyperdense lesion in the basal ganglia bilaterally, with the presence of right parietal epidural hematoma. Craniotomy and epidural hematoma drainage were considered, associated to conservative management of gangliobasal traumatic contusions. On day 7 the patient had sudden neurologic deterioration, cardiac arrest unresponsive to resuscitation. Management of these lesions is similar to any other injury in moderate to severe traumatic injury. The use of intracranial pressure monitoring must be guaranteed.