Delayed post-hypoxic leucoencephalopathy presenting akinetic mutism after traumatic cervical cord injury: a case report.

Delayed post-hypoxic leucoencephalopathy is a demyelinating syndrome characterized by acute onset of neuropsychiatric symptoms including parkinsonism or akinetic mutism that occurs days to weeks after recovery from cerebral hypo-oxygenation. We encountered a patient who presented with an akinetic mute state late after cervical cord injury without a definite hypoxic event.

Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele.

Combined spinal arteriovenous malformation and lipomyelomeningocele are extremely rare. We present a rare combined case of a lipomyelomeningocele with an intramedullary arteriovenous malformation (AVM) occurred at the L3-L4 level in a 30-year-old man who suffered from low back pain radiating to the lower extremities, dysuria, and frequency for 5 years. The MR studies showed an intradural mass with high-signal intensity on both T1-weighted and T2-weighted images, intermingled with multiple signal-void structures. The mass extended extradurally toward a subcutaneously forming fatty mass on the patient's back. Spinal angiography showed an AVM supplied by the radiculopial branches of the lumbar arteries and drained by tortuous, dilated, perimedullary veins. Endovascular embolization and surgical resection were performed.

Expression of interleukin-1 beta in lipopolysaccharide stimulated monocytes derived from patients with aneurysmal subarachnoid hemorrhage is correlated with cerebral vasospasm.

Cerebral arterial vasospasm is a frequent complication after aneurysmal subarachnoid hemorrhage (SAH). Immunological activation may affect the development of vasospasm. This study measured the cytokines released from lipopolysaccharide-stimulated monocytes of SAH patients. We measured cerebral artery diameters before and after surgery for SAH. The activation index of interleukin-1 beta (IL-1 beta), but not tumor necrosis factor-alpha (TNF-alpha), was higher in patients with symptomatic vasospasm (5.6+/-1.7; n=11) than in patients without (1.8+/-0.4; n=11) (P=0.039). Furthermore, the IL-1 beta activation index was correlated with the degree of the postoperative angiographic vasospasm (correlation coefficient=-0.66, P=0.007). Individual variation in systemic immune activation, measured by monocyte-derived IL-1 beta expression levels after stimulation, may be associated with the development of vasospasm after aneurysmal SAH.

Increased burst firing in substantia nigra pars reticulata neurons and enhanced response to selective D2 agonist in hemiparkinsonian rats after repeated administration of apomorphine.

Intermittent administrations of dopaminergic agents in hemiparkinsonian rat enhances the behavioral response to subsequent administration of the drugs. This phenomenon is known as "priming" and thought as comparable to drug-induced dyskinesia in patients with Parkinson's disease. We investigated the behavioral and electrophysiological changes in 6-hydroxydopamine (6-OHDA)-lesioned hemiparkinsonian rats after repeated administrations of apomorphine. Administration of apomorphine (0.32 mg/kg, intraperitoneal, i.p.) twice daily for 6 days enhanced the rotation induced by apomorphine from 341 turns/hour at the beginning to 755 turns/hr at the end. At the same time, the response to selective D2 agonist quinpirole (0.26 mg/kg, i.p.) was also enhanced from 203 to 555 turns/hr. Extracellular single unit recording revealed no significant difference in the basal firing rates of substantia nigra pars reticulata (SNr) neurons between the ipsilateral and contralateral side of the 6-OHDA lesion regardless of the repeated administrations of apomorphine. In SNr of the lesion side, the units with burst firing pattern were found more frequently after repeated administrations of apomorphine and the suppressive effect of quinpirole on the firing rate was enhanced. These findings suggest that the increased percentage of the burst units is the important electrophysiological change in the development of enhanced response to selective D2 agonist.

Clinical significance of molecular genetic changes in sporadic invasive pituitary adenomas.

Several molecular and genetic changes have been found in pituitary adenomas. We looked for correlations between these changes and the degree of invasiveness of the tumors. The invasiveness of 11 pituitary adenomas was graded by Hardy classification. We examined the retinoblastoma gene (RB1.20 on chromosome 13q) and the region around the MEN1 locus (chromosome 11q13.1-5) for loss of heterozygosity. Also examined are p53 mutations using single strain conformation polymorphism, p53 protein overexpression using immuno cytochemistry, homozygous deletions of p15 and p16 by polymerase chain reaction, and cellular proliferative activity using MIB-1 antibody. Six tumors (54.5%) had an LOH at either RB1.20 or the MEN1 locus. LOHs were found more frequently in Grade 4 and stage E tumors (72% and 67%) than in Grade 3 and stage D tumors (25% and 40%). However, no mutation or overexpression of p53 was found. No homozygous deletions of p15 or p16 were identified. The cell proliferative index ranged from 0 to 3%. LOH at 11q13 and 13q may be valuable in predicting the invasiveness of pituitary adenomas.

Surgical tactics and outcome of treatment in jugular foramen schwannomas.

Seven patients with schwannomas of the jugular foramen were included our study in Samsung Medical Center between 1995 and 1999. Patients with neurofibromatosis were excluded. The records of the seven patients (six surgical case and one nonsurgical case) were retrospectively reviewed. There were six women and one man (mean age, 47 years) with a symptom duration ranging from 3 months to 14 years (mean, 47 months). The predominant symptoms were hearing difficulty, hemifacial spasm and hoarseness. Preoperative audiologic evaluation, computerised tomography (CT), magnetic resonance (MR) imaging, and angiography were performed in most patients. We classified tumours into four types using Kaye and Pellet classification on the basis of radiological and surgical findings. The tumours were: Type A (at cerebellopontine angle) in one; Type B (foraminal) in two; Type C (extracranial and/or foraminal) in two; and Type D (intra- and extracranial) in two cases. We used various surgical approaches such as retrosigmoid suboccipital craniectomy for Type A tumours, infratemporal fossa type A approach (ITFA) for Type C tumours, petro-occipital transsigmoid approach or modified transcochear approach for Type D tumours and ITFA with partial labyrinthectomy for Type B. In the selection of surgical approaches, we took consideration of tumour extension, tumour size, and preoperative hearing function. Facial nerve transposition was not used only in one case of ITFA because of small tumour size (1.5cm). Gross total removal was achieved in five cases, and subtotal removal in one case (Type D tumour) with a single-stage operation. Stereotactic radiosurgery was performed on residual mass in the subtotally removed case. Follow-up period ranged from 13 to 49 months (mean, 27.5 months). There was neither postoperative mortality nor recurrence on follow-up MR imaging. There were two cases of temporary facial nerve palsy and one aggravation of pre-existing low cranial palsy. Two case of sustained vocal cord palsy underwent thyroplasty, but there was no aspiration pneumonia. Persistent cerebrospinal fluid collection was improved with lumboperitoneal shunt. The surgical approaches of each case should be tailored according to their shape and the clinical manifestation. We obtain acceptable outcomes from one-stage operation.

Surgical treatment of intractable epilepsy accompanying cortical dysplasia.

OBJECT: Surgical treatment of cortical dysplasia (CD) together with intractable seizures is challenging because both visualization and localization of the lesion are difficult, correlation with seizure foci requires comprehensive study, and the surgical outcomes reported thus far are unsatisfactory. The authors report their experience in the surgical treatment of CD classified according to a surgical point of view. METHODS: The definition of CD used in this study was a dysplastic lesion visible on magnetic resonance (MR) images or a lesion that, although not visible on MR images, was diagnosed as moderate-to-severe dysplasia by using pathological analysis. During the last 4.5 years, the authors treated 36 patients with intractable epilepsy accompanied by CD. They divided the 36 cases of CD into four characteristic groups: Group A, diffuse bilateral hemispheric dysplasia; Group B, diffuse lobar dysplasia; Group C, focal dysplasia; and Group D, a moderate to severe degree of CD with a normal appearance on MR images. All but one patient in Group C were monitored in the epilepsy monitoring unit by using subdural electrodes for seizure localization and functional mapping. The incidence of CD among a cohort of 291 patients who had undergone epilepsy surgery at the authors' center during the study period was 12.4%. The mean age of the 36 patients was 21.3 years and the mean age at seizure onset was 8.5 years. The mean follow-up period was 26 months. Twenty-six patients (72.2%) belonged to Engel Class I or II (20 and six, respectively). There were five cases in Group A, nine in Group B, nine in Group C, and 13 in Group D. Patients in Groups A and B were significantly younger at seizure onset and had significantly poorer surgical outcomes compared with patients in Groups C and D (p < 0.05). If outcome is compared on the basis of the extent of removal of CD, patients in whom CD was completely removed had significantly better outcomes than those in whom CD was only partially removed (p < 0.001). CONCLUSIONS: The authors conclude that intractable epilepsy accompanied by CD can be treated surgically using comprehensive preoperative approaches. Deliberate resective procedures aimed at complete removal of dysplastic tissue ensure excellent seizure control without permanent neurological deficit.

Vascular endothelial growth factor expression under ischemic stress in human meningiomas.

Vascular endothelial growth factor (VEGF) is an endothelial cell-specific antigen and angiogenic factor that plays a role in angiogenesis. We analyzed the expression of four VEGF mRNA isoforms in meningiomas. Among 35 meningiomas, 11 came from patients who underwent complete (n=4) or partial (n=7206=189 in all samples. However, the VEGF121 and 165 isoforms were significantly upregulated in samples from patients who underwent partial preoperative embolization. The diffusible VEGF121 isoform may be important for vascularity and edema formation in meningiomas.

Traumatic spinal subdural hematoma: rapid resolution after repeated lumbar spinal puncture and drainage.

A 15-year-old boy developed back pain and sciatica after a minor trauma. Magnetic resonance imaging revealed lumbar spinal subdural hematoma. After repeated lumbar spinal puncture and drainage of hemorrhagic fluid, spinal subdural hematoma was resolved completely. The benefits of conservative treatment by lumbar spinal puncture are discussed.