Isolated leptomeningeal angiomatosis in the sixth decade of life, an adulthood variant of Sturge Weber Syndrome (Type III): role of advanced Magnetic Resonance Imaging and Digital Subtraction Angiography in diagnosis.

BACKGROUND: Sturge-Weber syndrome (SWS) is primarily diagnosed in pediatric population, but clinical presentation in late adulthood is rarely reported. Evolution of radiological findings in the adulthood variant of SWS with isolated leptomeningeal angiomatosis has never been reported to our knowledge. CASE PRESENTATION: We report here a case of an isolated temporo-parieto-occipital leptomeningeal angiomatosis on the right cerebral hemisphere in a sixty-two-year-old male who presented with generalized seizure, GCS score 14/15 (E4 V4 M6) with equal and reacting pupils, psychomotor slowing, left hemineglect and grade 4 power in the left upper and lower limbs. Over a period of 48 h his neurological status deteriorated, but recovered spontaneously over a week on titration with anticonvulsants. He had a prior history of treatment for focal leptomeningitis, three years ago. Cerebrospinal fluid (CSF) analysis showed glucose of 75 mg/dL, proteins of 65 mg/dL and culture grew no organisms. On follow-up, he had intermittent episodes of focal seizure for two years. Initial, computed tomography of brain showed hyperdense lesion in the parieto-occipital convexity subarachnoid space on the right cerebral hemisphere mimicking subarachnoid hemorrhage and computed tomography angiography showed no significant abnormality. Magnetic resonance imaging (MRI) of brain showed intense pial enhancement in the right temporo-parieto-occipital region with a subtle T2W hyperintense signal in the underlying subcortical white matter without edema or infarct or mass effect. Digital subtraction cerebral angiography (DSA) showed hypertrophy of the cerebral arteries, arteriolo-capillary bed and venules in the right temporo-parieto-occipital territory associated with early arterio-capillary and venous opacification. Serial MRI done after six months, one and two years showed increase in the T2W hyperintense signal in the subcortical white matter and cortical atrophy with no changes in the pial enhancement. MR perfusion imaging showed reduced cerebral blood flow (CBF) and cerebral blood volume (CBV) in the right parieto-temporo-occipital cortical and subcortical regions and increased perfusion in the leptomeninges with reduction of the NAA / Cr ratios in spectroscopy. CONCLUSION: Conglomeration of various radiological findings in MRI, Perfusion, MRS and DSA with the clinical presentation can aid in establishing the diagnosis of this rare presentation of SWS-type 3 variant in late adulthood.

Accuracy of the freehand (fennell) technique using a uniform entry point and sagittal trajectory for insertion of thoracic pedicle screws: A computed tomography-based virtual simulation study.

BACKGROUND: Thoracic spine has complex pedicle anatomy with a narrow canal diameter which makes pedicle screw insertion challenging. Fennell et al. have described a simple freehand technique of thoracic pedicle screw placement. We have tested the accuracy of Fennell technique using computed tomography-based (CT-based) simulation model with pedicle screw simulator (PSS). METHODS: Normal CT thoracic spine obtained from CT thorax data of five patients were used in the 3D slicer environment using PSS for simulation. Entry points and axial trajectory as described by Fennell et al. and a sagittal trajectory parallel to the superior endplate were used for simulating the freehand technique using EA (entry angle) mode in the PSS. An ideal trajectory through the midsection of the pedicle from the same entry point and a sagittal trajectory parallel to the superior endplate were simulated using the ET (Entry Target) mode. Angle predicted by the software for an ideal axial trajectory was compared with the Fennell technique and this angle difference was noted at all the levels. Presence of pedicle breach was noted while simulating the Fennell technique. RESULTS: A total of 240 thoracic pedicle screw insertions were simulated, 120 screws by each technique. A sagittal trajectory parallel to the superior endplate caused no pedicle breach in the cranial-caudal direction at any level. No medial or lateral breach was noted while using an axial trajectory of 30° at T1-T2 and 20° from T3-T10. A 20° axial trajectory at T11 and T12 resulted in a breach of the medial cortex and the ideal mean axial angles at T11 and T12 were 2.8° and 6.5°, respectively. CONCLUSIONS: Fennell technique was effectively simulated using PSS. A uniform entry point and sagittal trajectory parallel to the superior endplate serves as a useful guide for freehand insertion of thoracic pedicle screws. At T11 and 12, ideal axial trajectories are less than 10°.

Changing trends of presentation of central nervous system tuberculosis: Relative prevalence of cranial and spinal tuberculosis and drug resistance patterns.

OBJECTIVE: Inappropriate use of antituberculosis drugs and a poor compliance has led to an increase in the prevalence of resistant Mycobacterium tuberculosis. The aim of this study was to document the changing trends in clinical presentation and drug resistance in patients with tuberculosis (TB) of the brain and the spine. MATERIALS AND METHODS: The authors retrospectively analyzed data from 243 patients admitted in a neurosurgical unit for surgical management of TB of the brain and spine from 2000 to 2013. To establish changes in trends, the patient population was arbitrarily divided into two groups based on their years of admission: 2000-2006 (Group A; n = 121) and 2007-2013 (Group B; n = 122). RESULTS: In the second era (Group B), there were 14.5% more patients with TB spine [from 42/121 (34.7%) in Group A to 60/122 (49.2%) in Group B; P = 0.02] with a corresponding reduction in the proportion of patients with TB brain. The number of cerebrospinal fluid (CSF) diversion procedures remained the same in both the groups, but there was significant reduction in other surgical procedures for patients with TB brain in Group B (P = 0.0004). In patients with TB brain, the culture yield was 10/50 (20%) from tissue and 8/72 (11%) from CSF and there was no significant difference between the groups. In patients with TB spine, the culture yield was higher in Group B patients but was not statistically significant [7/35 (20%) in Group A versus 18/57 (31.6%) in Group B (P = 0.27)]. In Group A, nine patients with TB brain grew Mycobacterium tuberculosis in culture and none was resistant to first-line antituberculosis therapy (ATT), while in Group B, nine patients grew the bacilli and five had resistance to first-line ATT (P = 0.03). Among patients with a positive culture of resistant TB, all had received prior ATT (100% secondary resistance). None of the seven patients with TB spine in Group A with a positive culture had resistant organisms, but in Group B, 5 of 18 (27.8%) with a positive culture had resistant organisms (P = 0.27). Of these, five patients with TB spine with resistance, three of five (60%) patients had secondary resistance, and two of five (40%) patients had primary resistance. Overall, 10 of 27 (37%) patients with a positive culture had resistant organisms in Group B, while none of 16 patients in Group A with a positive culture had resistant organisms (P = 0.007). CONCLUSION: The most significant finding of our study is an alarming increase in the number of patients with TB brain and spine who have resistant disease (from 0% to 37%) with most of the resistance being secondary in nature. There was an increase in the number of in-patients with spinal TB relative to those with TB brain, though the cause for this is unclear.

Patient-specific interactive software module for virtual preoperative planning and visualization of pedicle screw entry point and trajectories in spine surgery.

BACKGROUND: Lumbar pedicle screw insertion involves a steep learning curve for novice spine surgeons and requires image guidance or navigation. Small volume centers may be handicapped by the lack of cost-effective user-friendly tools for preoperative planning, guidance, and decision making. OBJECTIVE: We describe a patient-specific interactive software module, pedicle screw simulator (PSS), for virtual preoperative planning to determine the entry point and visualize the trajectories of pedicle screws. MATERIALS AND METHODS: The PSS was coded in Python for use in an open source image processing software, 3D Slicer. Preoperative computed tomography (CT) data of each subject was loaded into this module. The entry-target (ET) mode calculates the ideal angle from the entry point through the widest section of the pedicle to the desired target in the vertebral body. The entry-angle (EA) mode projects the screw trajectory from the desired entry point at a desired angle. The performance of this software was tested using CT data from four subjects. RESULTS: PSS provided a quantitative and qualitative feedback preoperatively to the surgeon about the entry point and trajectories of pedicle screws. It also enabled the surgeons to visualize and predict the pedicle breach with various trajectories. CONCLUSION: This interactive software module aids in understanding and correcting the orientation of each vertebra in three-dimensions, to identify the ideal entry points, angles of insertion and trajectories for pedicle screw insertion to suit the local anatomy.

Primary Intradural Extramedullary Cervical Spinal Cysticercosis.

BACKGROUND: Spinal cysticercosis has been reported in 0.7%-3.0% of patients with neurocysticercosis. Most patients with spinal cysticercosis have a coexisting intracranial disease. Most often this intracranial disease manifests as intradural extramedullary lesions involving thoracic and lumbar regions or intramedullary lesions. Intradural extramedullary primary spinal cysticercosis manifesting as cervical myelopathy is extremely rare and has not been reported to date. CASE DESCRIPTION: A 56-year-old man from the northeastern part of India presented with progressive spastic quadriparesis. Magnetic resonance imaging showed a ventrally located intradural extramedullary multiloculated cyst with an enhancing wall in the upper cervical region. Enzyme-linked immunoelectrotransfer blot performed to detect cysticercal antibodies in serum was positive. The patient underwent total excision of the cysts, which were confirmed histologically to be cysticercal cysts. He was also treated with 2 weeks of albendazole therapy after surgery. He had recovered fully 1 year later. CONCLUSIONS: Cysticercosis should be considered in the differential diagnosis in a patient with multiloculated cysts in the spinal subarachnoid space. Surgical exploration and excision of the cysts should be performed not only to establish a diagnosis but also to decompress the cord before medical therapy.