Tentorial Venous Anatomy: Variation in the Healthy Population.

BACKGROUND AND PURPOSE: A new transtentorial venous system consisting of medial, intermediate, and lateral tentorial veins, connecting infra- and supratentorial compartments, was recently shown in 2 cadaver dissections and 2 patient scans. We sought to characterize the venous patterns within the tentorium and their relation to measures of skull development in a cohort of healthy adults. MATERIALS AND METHODS: We retrospectively reviewed tentorial venous anatomy of the head using CTA/CTV performed for routine care or research purposes in 238 patients. Included studies had adequate contrast opacification of venous structures and a section thickness of ≤2 mm; we excluded cases with space-occupying lesions and vascular pathologies. Tentorial angle, dural sinus configurations, and measures of skull base development were assessed as predictors of tentorial venous anatomy variation via Cramér V association, the binary encoded Pearson correlation, and nearest-point algorithm with the Euclidean distance metric for clustering. RESULTS: Tentorial vein development was related to the ringed configuration of the tentorial sinuses (P < .005). There were 3 configurations. Groups 1A and 1B (n = 50/238) had ringed configuration, while group 2 did not (n = 188/238). Group 1A (n = 38/50) had a medialized ringed configuration, and group 1B had a lateralized ringed configuration (n = 12/50). Measurements of skull base development were predictive of these groups. The ringed configuration of group 1 was related to the presence of a split confluens, which correlated with a decreased internal auditory canal-petroclival fissure angle. Configuration 1A was related to the degree of petrous apex pneumatization (P value = .010). CONCLUSIONS: Variations in the transtentorial venous system directly correlate with cranial development.

[The role of Integrating the Healthcare Enterprise (IHE) in telemedicine]. / Die Rolle von Integrating the Healtcare Enterprise (IHE) in der Telemedizin.

BACKGROUND: Telemedicine systems are today already used in a variety of areas to improve patient care. The lack of standardization in those solutions creates a lack of interoperability of the systems. Internationally accepted standards can help to solve the lack of system interoperability. With Integrating the Healthcare Enterprise (IHE), a worldwide initiative of users and vendors is working on the use of defined standards for specific use cases by describing those use cases in so called IHE Profiles. OBJECTIVES: The aim of this work is to determine how telemedicine applications can be implemented using IHE profiles. METHODS: Based on a literature review, exemplary telemedicine applications are described and technical abilities of IHE Profiles are evaluated. These IHE Profiles are examined for their usability and are then evaluated in exemplary telemedicine application architectures. RESULTS: There are IHE Profiles which can be identified as being useful for intersectoral patient records (e.g. PEHR at Heidelberg), as well as for point to point communication where no patient record is involved. In the area of patient records, the IHE Profile "Cross-Enterprise Document Sharing (XDS)" is often used. The point to point communication can be supported using the IHE "Cross-Enterprise Document Media Interchange (XDM)". IHE-based telemedicine applications offer caregivers the possibility to be informed about their patients using data from intersectoral patient records, but also there are possible savings by reusing the standardized interfaces in other scenarios.

"Hidden" bone metastasis from thyroid carcinoma: a clinical note.

The (131)I-iodide ((131)I) whole-body scan, for thyroid carcinoma is at times difficult to interpret. In a diagnostic whole body (131)I scan of a patient with follicular carcinoma, a posterior skull lesion was partially hidden by overlapping facial structures. On lateral head view, the abnormality was clearly evident. SPECT/CT and MRI showed the lesion originated in the occipital bone and had enlarged into the posterior fossa. The mass was surgically removed and the patient received (131)I therapy for residual tissue. The study demonstrates a pitfall in the reading of two dimensional radioiodine images which can be overcome by SPECT or lateral imaging.

Peripheral and central hypomyelination with hypogonadotropic hypogonadism and hypodontia.

We identified four unrelated patients (three female, one male) aged 20 to 30 years with hypomyelination, pituitary hypogonadotropic hypogonadism, and hypodontia. Electron microscopy and myelin protein immunohistochemistry of sural nerves showed granular debris-lined clefts, expanded abaxonal space, outpocketing with vacuolar disruption, and loss of normal myelin periodicity. Reduced galactocerebroside, sphingomyelin, and GM1-N-acetylglucosamine and increased esterified cholesterol were found. This is a clinically homogeneous progressive hypomyelinating disorder. The term 4H syndrome is suggested.

[Dynamic MRI of the bone marrow for monitoring multiple myeloma during treatment with thalidomide as monotherapy or in combination with CED chemotherapy]. / Dynamische MRT des Knochenmarks zum Monitoring des Multiplen Myeloms unter Thalidomid-Monotherapie oder Kombination mit CED-Chemotherapie.

PURPOSE: To quantify changes of bone marrow microcirculation in multiple myeloma (MM) using contrast enhanced dynamic MRI (dMRI) during thalidomide as antiangiogenic monotherapy or in combination with chemotherapy (cyclophosphamide, etoposide, dexamethasone). MATERIALS AND METHODS: The study includes 63 patients with refractory or relapsed MM, who underwent dMRI with high temporal resolution (T1w-turboFLASH) of the lumbar spine before and following treatment. The contrast uptake was quantified using a two compartment model with the output parameters amplitude and k (ep) (exchange rate constant). The evaluation considered the initial dMRI finding (pathological or non-pathological) and the clinical therapeutic response (response or no response). RESULTS: During monotherapy with thalidomide (n = 38), no significant changes of the dMRI parameters were found, even when considering the initial dMRI finding (positive n = 22) and the therapeutic response (responder n = 14). The combination with chemotherapy (n = 25) had a significant reduction of k (ep) (p = 0.01) in 18 patients with positive initial dMRI finding and therapeutic response. Reduction of the amplitude was seen in most cases, but in the end without any significance (p = 0.09). CONCLUSION: dMRI can quantify significant changes of bone marrow microcirculation solely during treatment with thalidomide combined with chemotherapy, not with thalidomide alone.

Clinical and neuroimaging features of "idiopathic" syringomyelia.

In some adult patients with cervical syringomyelia, MRI studies do not identify primary disease within the foramen magnum or spinal canal. To identify the etiology of this idiopathic type of syringomyelia, clinical features and posterior fossa (PF) measurements from 17 of these patients, 17 patients with Chiari I-type syringomyelia, and 32 control subjects were compared. Idiopathic syringomyelia and Chiari I-type syringomyelia manifested central cervical myelopathy and a small PF with narrow CSF spaces, suggesting that they develop by the same mechanism.

Detection of human herpesvirus-6 in mesial temporal lobe epilepsy surgical brain resections.

BACKGROUND: Human herpesvirus-6 (HHV-6), a ubiquitous beta-herpesvirus, is the causative agent of roseola infantum and has been associated with a number of neurologic disorders including seizures, encephalitis/meningitis, and multiple sclerosis. Although the role of HHV-6 in human CNS disease remains to be fully defined, a number of studies have suggested that the CNS can be a site for persistent HHV-6 infection. OBJECTIVE: To characterize the extent and distribution of HHV-6 in human glial cells from surgical brain resections of patients with mesial temporal lobe epilepsy (MTLE). METHOD: Brain samples from eight patients with MTLE and seven patients with neocortical epilepsy (NE) undergoing surgical resection were quantitatively analyzed for the presence of HHV-6 DNA using a virus-specific real-time PCR assay. HHV-6 expression was also characterized by western blot analysis and in situ immunohistochemistry (IHC). In addition, HHV-6-reactive cells were analyzed for expression of glial fibrillary acidic protein (GFAP) by double immunofluorescence. RESULTS: DNA obtained from four of eight patients with MTLE had significantly elevated levels of HHV-6 as quantified by real-time PCR. HHV-6 was not amplified in any of the seven patients with NE undergoing surgery. The highest levels of HHV-6 were demonstrated in hippocampal sections (up to 23,079 copies/10(6) cells) and subtyped as HHV-6B. Expression of HHV-6 was confirmed by western blot analysis and IHC. HHV-6 was co-localized to GFAP-positive cells that morphologically appeared to be astrocytes. CONCLUSIONS: HHV-6B is present in brain specimens from a subset of patients with MTLE and localized to astrocytes in the absence of inflammation. The amplification of HHV-6 from hippocampal and temporal lobe astrocytes of MTLE warrants further investigation into the possible role of HHV-6 in the development of MTLE.

Polysomnographic pattern recognition for automated classification of sleep-waking states in infants.

A robust, automated pattern recognition system for polysomnography data targeted to the sleep-waking state and stage identification is presented. Five patterns were searched for: slow-delta and theta wave predominance in the background electro-encephalogram (EEG) activity; presence of sleep spindles in the EEG; presence of rapid eye movements in an electro-oculogram; and presence of muscle tone in an electromyogram. The performance of the automated system was measured indirectly by evaluating sleep staging, based on the experts' accepted methodology, to relate the detected patterns in infants over four months of post-term age. The set of sleep-waking classes included wakefulness, REM sleep and non-REM sleep stages I, II, and III-IV. Several noise and artifact rejection methods were implemented, including filters, fuzzy quality indices, windows of variable sizes and detectors of limb movements and wakefulness. Eleven polysomnographic recordings of healthy infants were studied. The ages of the subjects ranged from 6 to 13 months old. Six recordings counting 2665 epochs were included in the training set. Results on a test set (2,369 epochs from five recordings) show an overall agreement of 87.7% (kappa 0.840) between the automated system and the human expert. These results show significant improvements compared with previous work.

Root and spinal cord compression from methylmethacrylate vertebroplasty.

STUDY DESIGN: Case report and literature review. OBJECTIVES: Clinicians use methylmethacrylate vertebroplasty to treat vertebral hemangiomas, metastases, and osteoporotic fractures. Cement may leak out of the vertebral body and compress the adjacent spinal cord and nerve roots. We review a case of nerve-root and cord compression from methylmethacrylate extrusion during vertebroplasty. SUMMARY OF BACKGROUND DATA: A 50-year-old female presented with disabling thoracic back pain. A metastasis to T1 was discovered, with collapse of the vertebral body but without cord compression. Methylmethacrylate vertebroplasty was performed. After injection, portable computed tomography (CT) showed a leakage of methylmethacrylate into the C8 and T1 foramina and spinal canal. Radiculopathy and myelopathy developed. Surgical decompression using the anterior approach was necessary. METHODS: Case report. RESULTS: Early surgical intervention decompressed the neural elements and relieved the neurological deficits. CONCLUSIONS: Neurologic complications of methylmethacrylate vertebroplasty necessitate active involvement of spine surgeons in patient evaluation and management.

Retrospective analysis of surgical treatment outcomes for gelastic seizures: a review of the literature.

Gelastic seizures are known to be refractory to medical treatment and to date surgical therapy has yet to pinpoint the best treatment for these refractory seizures. There has been a multitude of case reports published on gelastic seizures and different surgical treatments, thus we performed a review of the literature on gelastic seizures and surgical treatments to elucidate the best surgical approaches for medically refractory gelastic seizures.

Symptomatic vertebral hemangiomas: treatment by means of direct intralesional injection of ethanol.

PURPOSE: To describe the technique and results of injecting ethanol directly into symptomatic vertebral hemangiomas. MATERIALS AND METHODS: Eleven patients with paraplegia (n = 6) or radiculopathy (n = 5) due to vertebral hemangioma were treated by means of injecting ethanol (5-50 mL) directly into the lesion with computed tomographic (CT) guidance. CT angiograms were essential prior to treatment to identify functional vascular spaces of the hemangioma and direct needle placement. RESULTS: All hemangiomas were obliterated completely at follow-up angiography and gadolinium-enhanced magnetic resonance imaging. Five of six patients with paraplegia recovered completely: One who was treated recently was walking with assistance. Four of five patients with radiculopathy improved. No immediate complications were associated with ethanol injection. The two patients who received the largest volumes of ethanol, 42 and 50 mL, developed pathologic fractures of the involved vertebrae 4 and 16 weeks after treatment. CONCLUSION: Direct injection of ethanol into symptomatic vertebral hemangioma is an effective and safe treatment, provided the dose is less than 15 mL.

Spinal cord swelling preceding syrinx development. Case report.

The pathophysiology of syrinx development is controversial. The authors report on a patient with progressive cervical myelopathy and a Chiari I malformation in whom spinal cord swelling preceded, by a few months, the development of a syrinx in the same location. The patient underwent a craniocervical decompressive procedure and duraplasty, and complete resolution of cord swelling and syringomyelia was achieved. This report is consistent with the theory that patients with Chiari I malformation have increased transmural flow of cerebrospinal fluid, which causes spinal cord swelling that later coalesces into a syrinx. The pathophysiology of syrinx development from spinal cord edema and the success of surgical decompressive treatments that do not invade the central nervous system support the prompt treatment of patients with spinal cord edema who are at risk for the development of a syrinx.

Elucidating the pathophysiology of syringomyelia.

OBJECT: Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy. METHODS: The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing. Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressure and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape. CONCLUSIONS: The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-I laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

A human bite.

We report the transmission of group A streptococci by a human bite leading to severe necrotising fasciitis. Rapid surgical and antibiotic treatment led to healing without fractional loss of the patient's infected leg.

Brain metastasis after immunotherapy in patients with metastatic melanoma or renal cell cancer: is craniotomy indicated?

The purpose of this study was to evaluate the outcome of surgical treatment of brain metastasis in patients with metastatic melanoma or renal cell cancer after interleukin-2 (IL-2) therapy. A retrospective analysis was conducted at the Surgery Branch, National Cancer Institute. All patients with a diagnosis of metastatic melanoma or renal cell cancer who received IL-2 from January 1, 1985 to January 1, 1996 (n = 1385) were screened for the development of brain metastasis. Forty patients underwent surgical treatment of brain metastasis that developed after initiating IL-2 therapy. Thirty-six were rendered free of disease after resection of a single metastasis and were the focus of this study. Twenty-two of the 36 patients achieved a clinical response (10 complete responses and 12 partial responses) at extracranial sites of disease after IL-2-based immunotherapy and before the development of brain metastasis. The median disease-free interval in the brain after resection of a single metastasis was 21, 7, and 3 months for patients achieving a complete response, partial response, and no response (CR, PR, and NR) to IL-2 therapy, respectively. The median survival after craniotomy for these three groups of patients was 23, 17, and 7 months, respectively. The disease-free interval in the brain and the overall survival after craniotomy were significantly longer for patients achieving a CR to previous immunotherapy when compared with patients achieving a PR or NR. Of the 10 patients who had achieved a prior CR, 8 remained disease free in the brain at last follow-up, 6 remained alive beyond 1 year, and 3 > 4 years. Twenty-five patients experienced neurologic symptoms before craniotomy and all had complete resolution of their symptoms after surgery. Surgical treatment of single brain metastasis in patients with metastatic melanoma or renal cell cancer is indicated in carefully selected patients. The benefits of resection include palliation of symptoms and the potential for a prolonged disease-free interval in the brain.