Faculty Bibliography

Musicogenic epilepsy (ME) is a condition in which seizures are triggered by music. We describe a novel investigation of the mechanisms of ME using noninvasive (ictal 2-flurodeoxyglucose (2-FDG) positron emission tomography (PET)) and invasive (subdural arrays and depth electrodes) methodology in one patient with ME. Ictal PET defined onsets in the right mesial temporal lobe. This was confirmed by intracranial electrode recordings, where there was onset in the right mesial temporal lobe, followed by sequential spread to the lateral temporal cortex, Heschel's gyrus, insula, and frontal lobes. Right temporal lobectomy resulted in Engel class 1 outcome

BACKGROUND: Spontaneous intracranial hypotension is an increasingly recognized cause of new-onset, daily, persistent headaches. Although these headaches are similar to post-lumbar puncture headaches, characteristic differences include intracranial pachymeningeal enhancement, subdural fluid collections, and downward displacement of the brain. The identification of upper cervical epidural fluid collections as a false localizing sign in patients with SIH has provided significant insight into the selection of management options. CASE DESCRIPTION: We review a case of a 57-year-old woman who presented to our institution with progressive orthostatic headaches relieved by recumbency. The patient had no recent history of lumbar puncture, spinal, or intracranial procedure. The patient isolated the onset of symptoms to 3 weeks prior, when she was lifting heavy items in her home, and was diagnosed with SIH. Subsequently, she was found to have a C1-C2 epidural fluid collection. After much diagnostic consideration and review of the literature, the collection was defined as a false localizing sign; and the patient eventually underwent a lumbar EBP with complete resolution of her symptoms. CONCLUSIONS: Upper cervical fluid collections in patients with SIH often represent a false localizing sign. Conservative management should be instituted; and if unsuccessful, a lumbar EBP should be performed. It is important to note that C1-C2 fluid accumulations may not provide the actual leak site in patients presenting with SIH. This understanding prevents therapy from being unintentionally directed at C1-C2

BACKGROUND: Patients with pineal lesions typically present with the classical signs of increased intracranial pressure (headache, nausea, vomiting) and/or Parinaud's syndrome. Rare symptomatology of secondary parkinsonism attributed to pineal lesions has been previously reported in the literature. We describe an unusual case of a pineal cyst in a patient with the presenting sign of a resting tremor. CASE DESCRIPTION: We report an 18-year-old Caucasian female who presented with a 1-month history of a new-onset progressive, unilateral low-frequency right-hand resting tremor with associated headache, nausea, vomiting, and excessive diarrhea. Magnetic resonance imaging demonstrated an atypical appearance with enhancement of a mildly prominent pineal gland, possibly representing a pineal cyst. The patient did not exhibit radiographic signs of hydrocephalus. Based upon the radiographic appearance, one could not exclude with absolute certainty the presence of a malignancy. The patient ultimately underwent a bilateral suboccipital craniotomy with gross total resection of the lesion. Postoperatively, the patient exhibited immediate resolution of her preoperative resting tremor and continues to be symptom free at 1 year. CONCLUSIONS: A new-onset, resting tremor and/or other secondary parkinsonism symptoms should raise clinical suspicions of pineal lesions. Treatment can be guided based on tissue type and the presence or absence of hydrocephalus. We observed that complete surgical resection of the lesion provided the best treatment option for the total resolution of symptoms attributed to the disturbance of the microvasculature surrounding the nigro-striatal-pallidal system

Astroblastoma, a unique glial tumor, has been described in the literature in case reports and small series. Its rarity has prevented evidenced-based treatment stratification. An 8-year-old boy presented with signs and symptoms of raised intracranial pressure. Imaging studies of the brain demonstrated a large heterogeneously enhancing solid mass in the left frontal lobe with punctate calcifications and cystic components that created subfalcine and uncal herniation. After tumor resection, histological diagnosis revealed a high-grade cerebral astroblastoma. The child's postoperative recovery was complicated by hydrocephalus that necessitated placement of a ventriculoperitoneal shunt. Fifty-four months after undergoing gross total resection and adjuvant radiation therapy, the patient, now 12-years-old, remains tumor free and neurologically stable. Chemotherapy was reserved in the event of tumor recurrence. Since the first description of astroblastoma, its histopathological and clinical features have been debated. We review the histology, immunohistochemistry, and cytogenetics of astroblastoma as well as examine the current literature and treatment strategies for the management of both low and high-grade tumors. Continued clinical studies and longer patient follow-up with a tumor based registry would further clarify optimal treatment for this rare tumor

Trigonocephaly accounts for approximately 10% of all craniosynostosis. Severe trigonocephaly results in a triangular-shaped forehead, superior-lateral orbital depression, hypotelorism, and compensatory occipital-parietal calvarial changes. Radiographic findings include ovoid orbits with parallel medial borders, thickened keel-shaped frontal bone, small ethmoid sinuses, and a short anterior cranial fossa with pitched sphenoid wings. Our experience with 50 infantile cases of severe nonsyndromic trigonocephaly patients treated from 1987 to 2005 is clinically reviewed to assess long-term growth based on a standardized operative technique. The average age of the patients at surgery was 6 months and the mean follow-up was 12 years. Our complication rate was 2%; the reoperative rate was 12%. The use of resorbable rigid plate fixation combined with alloplastic augmentation has improved the cosmetic outcome in patients treated since 1996 and reduced the reoperative rate

Crouzon's disease is a well-known disorder affecting multiple organ systems, specifically a craniofacial disorder with highly variable penetrance and severity of deformity. Crouzon's patients typically have anomalies of the skull base leading to gross distortion of the cranium and in some cases the cervicocranium. We present a 5-year-old girl with Crouzon's disease who suffered from an acquired Chiari I malformation after insertion of a ventriculoperitoneal shunt and a coexistent ventral odontoid panus. Both these lesions were causing cervicomedullary compression. The literature is controversial on the surgical management of anterior and posterior compression at the craniocervical junction. We review the literature on surgical options for decompression at the craniocervical junction and offer our surgical case as a treatment option for patients in this rare clinical situation

Norian CRS, Bone Cement (Synthes CMF), and Mimix (Lorenz) have been used to reconstruct large cranio-orbital defects in 85 patients. Resorbable mesh (Macropore), used in combination in selected patients, obviates dura pulsations that have been postulated to cause fragmentation of alloplastic material. Norian is composed of monocalcium phosphate, monohydrate, alpha-tricalcium phosphate, and calcium carbonate. Admixture with NaPO4 creates dahllite, which has a higher carbonate content (4%--6%) than hydroxyapatite (0%). CRS is soluble at low pH, facilitating its resorption and replacement by bone. In contradistinction, Mimix is converted to aqueous solution at 37 degrees C, supports fibrovascular ingrowth and bony interdigitation at the implant-material surface. Forty-five adults (mean age=42 years) and 40 children (mean age=8 years) were evaluated with respect to etiology of defect, size, location, gram usage of alloplast material, type of alloplast, postoperative clinical course, and complications. A minimum of 3-year follow-up is available; 22 adult patients additionally underwent resorbable mesh reconstruction. There were 7 (8%) complications, including infection, extrusion, a sterile loculated fluid collection and fragmentation. No difference in complication rate was noted between biomaterials. Two additional patients exhibited resorption (Norian), necessitating reaugmentation. Alloplastic replacement of cranio-orbital defects has recently advanced dramatically. Bivalved cranial bone grafting with its attached morbidity and sequelae can be avoided. Resorbable mesh allows for the placement of alloplast material in larger defects while avoiding dura pulsation causing alloplast fragmentation. In avoiding titanium type reconstruction, it obviates any interference with radiologic diagnosis and radiotherapeutic modalities. Long-term results are needed to assess bone growth within alloplast and to study bone growth in alloplastic reconstructed pediatric patients

To provide the neurological and neurosurgical communities with case evidence of postoperative multiple sclerosis (MS) relapse, literature review to support operative stress-induced relapse and recommendations for perioperative prophylaxis to prevent relapse in patients undergoing surgery. Two case studies are presented with recommendations based on an extensive review of the medical literature and personal experience to support perioperative prophylactic suggestions. Both patients fully recovered to preoperative functional status after treatment. We now routinely implement perioperative prophylaxis to MS patients undergoing surgery at our institution with no complications to date. Perioperative prophylaxis in patients with MS undergoing surgery can prevent relapse. It is of utmost importance that the surgical community realizes that prophylactic treatment is available and should be utilized during elective and emergent surgical situations

Accelerated development of secondary sexual characteristics or sexual precocity is a well-known entity. Most authors recognize two groups of patients, those described as having central precocious puberty (CPP) and those with precocious pseudopuberty. CPP results from premature activation of the hypothalamic-pituitary-gonadal axis and pseudopuberty is caused by lesions that secrete gonadotropin-like substances or hormones. The onset of CPP is usually before age 8 in females and age 9 in males; however, there is contention that the age of onset is much earlier and also differs depending on the patients' race. Previously reported causes of CPP include intracranial neoplasm, infection, trauma, hydrocephalus and Angelman's syndrome. Pineal cysts are usually asymptomatic incidental findings, but have been associated with CPP. We present an interesting case of a patient with CPP and an associated pineal cyst. We review the literature on the pathogenesis of CPP and associated pineal cyst, the neuroendocrine relationship between the pineal gland and puberty and the neurosurgical role in these cases

Postoperative complications from corticosteroids in neurosurgical patients are not uncommon. Too often the deleterious immunosuppressive effects of corticosteroids are overlooked in neurosurgery patients and can lead to serious and lethal infections. EXPERIMENTAL DESIGN: case report of a 16-year-old healthy male who presented for elective resection of a recurrent juvile pilocytic astrocytoma of the posterior fossa 4 years after initial resection. SETTING: major University institutional practice. INTERVENTION/RESULTS: a standard suboccipital craniotomy with gross total resection. Postoperatively, the patient suffered from posterior fossa syndrome and diminished gag reflex requiring nasogastric feeds with progressive improvement. While awaiting transfer to a rehabilitation center on postoperative day 12 he suffered a sudden temperature spike followed by neurological decline. A stat computed tomography scan of the brain revealed a diffuse miliary process with severe cerebral edema. Sputum and cerebrospinal fluid cultures identified Aspergillus. Despite immediate therapy to combat the malignant cerebral edema, the patient died within 24 hours of onset of the symptoms. Corticosteroids are used routinely in neurosurgery to combat cerebral edema without much consideration for the immunosuppressive effects. This case demonstrates how the immunosuppressive effects of corticosteroids can lead to a fulminant lethal fungal infection. Neurosurgeons should be aware of the anticatabolic medications now available to combat the deleterious side effects of corticosteroids