Epilepsy Outcomes and Hippocampal Volumes in Children with Multiple Parenchymal Neurocysticercoses.

Background: We aimed to study the radiological and epilepsy outcomes of children with multiple neurocysticercosis (NCC) and the relationship with hippocampus volume. Study Design: Observational study. Materials and Methods: Children between 5 and 18 years of age with NCC lesions and completed more than 2-year follow-up were enrolled. Magnetic resonance imaging (MRI) brain with a predefined protocol was done as per clinical indication, and bilateral hippocampi volume was measured with a region of interest-based manual method. Proportion of children who had radiological resolution of lesions, proportion of children who were seizure-free for at least 3 months after antiepileptic drugs withdrawal, and mean hippocampi volume were calculated. Results: During the study period, 229 children followed up in pediatric NCC clinic were enrolled, the mean age was 10.5 ± 3 years, and 129 (56%) were boys. A total of 159 (65%) children had single-lesion NCC, and 79 (35%) had multiple lesion (> 2) NCC. Resolution of lesions was seen in 8% children with multiple NCC, significantly less as compared to the single NCC group (8% vs. 27%, P = 0.006). Multiple-lesion NCC group had a higher rate of calcification as compared to children with a single lesion (66% vs. 47%, P = 0.008). Twenty-nine (37%) children with multiple-lesion NCC were seizure free for at least 3 months after antiepileptic drug withdrawal. Mean combined hippocampal volume in children with single-lesion NCC was 2.96 (0.62) mL and multiple-lesion NCC was 2.88(0.43) mL. Conclusion: Children with multiple-lesion NCC have lower resolution, and higher calcification during follow-up in comparison to single-lesion NCC. Frequency of children who remained seizure-free 3-month post AED withdrawal was similar in single- and multiple-lesion groups.

Bone density and bone health alteration in boys with Duchenne Muscular Dystrophy: a prospective observational study.

OBJECTIVES: Boys with Duchenne Muscular Dystrophy (DMD) are at increased risk for compromised bone health, manifesting as low-impact trauma long bone fractures and vertebral compression fractures. METHODS: In a prospective observational study, we studied bone health parameters in North Indian boys with DMD. We consecutively enrolled ambulatory boys with DMD on glucocorticoid therapy. Bone health was evaluated with X-ray spine, Dual-energy X-ray absorptiometry (DXA), serum calcium, vitamin D3 (25[OH]D), 1,25-dihyroxyvitamin D3 (1,25[OH]2D3), serum osteocalcin, osteopontin, and N terminal telopeptide of type 1 collagen (Ntx) levels. RESULTS: A total of 76 boys with DMD were enrolled. The median age was 8.5 (interquartile range [IQR] 7.04-10.77) years. Among these, seven (9.2%) boys had long bone fractures, and four (5.3%) had vertebral compression fractures. Fifty-four (71%) boys underwent DXA scan, and among these 31 (57%) had low bone mineral density (BMD, ≤-2 z-score) at the lumbar spine. The mean BMD z-score at the lumbar spine was -2.3 (95% confidence interval [CI] = -1.8, -2.8), and at the femoral neck was -2.5 (95% CI = -2, -2.9). 25(OH)D levels were deficient in 68 (89.5%, n=76) boys, and 1,25(OH)2D3 levels were deficient in all. Mean serum osteocalcin levels were 0.68 ± 0.38 ng/mL (n=54), serum osteopontin levels were 8.6 ± 4.6 pg/mL (n=54) and serum Ntx levels were 891 ± 476 nmol/L (n=54). Boys with low BMD received glucocorticoids for longer duration, in comparison to those with normal BMD (median, IQR [16.9 (6-34) months vs. 7.8 (4.8-13.4) months]; p=0.04). CONCLUSIONS: Bone health is compromised in North Indian boys with DMD. BMD at the lumbar spine is reduced in more than half of boys with DMD and nearly all had vitamin D deficiency on regular vitamin D supplements. Longer duration of glucocorticoid therapy is a risk factor for low BMD in our cohort.

Long-term Clinical and Radiologic Outcome in 500 Children With Parenchymal Neurocysticercosis.

BACKGROUND: Our aim was to study long-term clinical and radiologic outcome in children with parenchymal neurocysticercosis (NCC) and its predictors. METHOD: Five hundred children with NCC registered in the pediatric NCC clinic between January 1996 and December 2002 and followed till December 2009 were enrolled. Demographic details, clinical presentations and therapy received were recorded. Outcome was evaluated in terms of seizure recurrence and resolution of lesions on neuroimaging. Various factors that could influence outcome were studied. RESULTS: Mean age at presentation was 8 ± 2.7 years, and the mean duration of follow-up was 8.8 ± 2.03 years; 79.6% (398) had single lesion, and 20.4% (102) had multiple lesions at presentation; 14.5% (58) of children with single lesion, and 28.4% (29) of children with multiple lesions had recurrent seizures (P < 0.001) during follow-up. At 6-month follow-up neuroimaging, resolution was seen in 52.7% (210) and 31.3% (32) of children with single and multiple lesions, respectively (P < 0.001). On prolonged follow-up, 94.6% (384) of single-lesion NCC and 88% (90) of multiple-lesion NCC (P < 0.001) had radiologic resolution. Single-lesion NCC, radiologic resolution and cysticidal therapy were associated with better seizure outcome (P < 0.05). Children with multiple lesions had significantly higher percentage of calcified lesions on long-term follow-up compared with those with a single lesion (11.7% vs. 3.6%, P < 0.05). CONCLUSIONS: Children with a single-lesion NCC have favorable outcome with resolution of most of the lesions and few seizure recurrences. Cysticidal therapy leads to better seizure control and increased resolution of lesions on short-term follow-up.

Revisiting the differences between irreducible and reducible atlantoaxial dislocation in the era of direct posterior approach and C1-2 joint manipulation.

OBJECTIVE The current management of atlantoaxial dislocation (AAD) focuses on the C1-2 joints, commonly approached through a posterior route. The distinction between reducible AAD (RAAD) and irreducible AAD (IrAAD) seems to be less important in modern times. The roles of preoperative traction and dynamic radiographs are questionable. This study evaluated whether differentiating between the 2 groups is important in today's era. METHODS Ninety-six consecutive patients with congenital AAD (33 RAAD and 63 IrAAD), who underwent surgery through a posterior approach alone, were studied. The preoperative and follow-up clinical statuses for both groups were studied and compared using Japanese Orthopaedic Association (JOA) scores. The radiological findings of the 2 groups were compared, and the intraoperative challenges described. RESULTS A poor preoperative JOA score (clinical status) was seen in one-fifth of patients with IrAAD, although the mean JOA score was nearly similar in the RAAD and IrAAD groups. There was significant improvement in follow-up JOA score in both groups. However, segmentation defects (such as an assimilated arch of the atlas and C2-3 fusion) and anomalous vertebral arteries were found significantly more often in cases of IrAAD compared with those of RAAD. Os odontoideum was commonly seen in the RAAD group. The C1-2 joints were acute in IrAAD compared with RAAD. Preoperative traction in IrAAD resulted in vertical distraction and improvement in clinical and respiratory status. Surgery for IrAAD required much more drilling and manipulation of the C1-2 joints while safeguarding the anomalous vertebral artery. CONCLUSIONS Bony and vascular anomalies were much more common in patients with IrAAD, which made surgery more challenging than it was in RAAD despite similar approaches. An irreducible dislocation seen on preoperative radiographs made surgeons aware of difficulties that were likely to be encountered and helped them to better plan the surgery. Distraction achieved through preoperative traction reaffirmed the feasibility of intraoperative reduction. This made the differentiation between the 2 groups and the use of preoperative traction equally important.

Delayed supratentorial intracerebral hemorrhage following posterior fossa surgery.

Delayed supratentorial intracerebral hematoma after posterior fossa surgery is uncommon. Only few cases have been reported in the past. The cause has been attributed to sitting position leading to changes in intracranial arterial and venous pressures. We report two cases of delayed intracerebral hematoma following posterior fossa surgery, none of which were operated in sitting position. MR venogram done in one patient showed venous sinus thrombosis. Intracererbal hematoma following infratentorial surgery is uncommon and is possibly due to venous sinus thrombosis leading to venous hypertension. Control of bleeding from venous sinuses due to avulsion of emissary veins during craniotomy/craniectomy possibly induces sinus thrombosis that may propagate antegrade or retrograde, leading to venous hypertension and parenchymal bleed.

Redefining Congenital Atlantoaxial Dislocation: Objective Assessment in Each Plane Before and After Operation.

BACKGROUND: The C1-C2 joint has multiple degrees of freedom of movement and C1-C2 dislocation (AAD) is often multiplanar. The existing methodology to assess the dislocation is limited to few planes. The object of this study is to redefine and objectively assess congenital AAD in each possible plane, before and after the operation. METHODS: This study consisted of 95 patients of irreducible congenital AAD operated on with the posterior approach alone. Preoperative and postoperative computed tomography imaging was studied in the axial, coronal, and sagittal planes. The relationship of C1-C2 along with the C1-C2 joint inclination was studied in each plane. The extent and type of dislocation was objectively assessed in each plane (newer indices) and compared with follow-up imaging for correction. The preoperative and postoperative Japenese orthopaedics association scores were compared. RESULTS: The commonest variety (61 patients) was a combination of anteroposterior (AP) and vertical C1-C2 dislocation. Five patients had predominant APnteroposterior, 6 vertical, 4 axial rotational, 9 lateral angular tilt, and 3 had lateral transalational. Seven patients had a combination of dislocation in AP, vertical, and rotational planes. AP dislocation was seen with sagittal inclination of C1-C2 joints and vertical dislocation with coronal inclination. Asymmetry in the joint's sagittal inclination added to a rotational component, whereas asymmetry in the coronal angulation caused lateral angular tilt. Pure rotational or lateral translation dislocation had near-normal C1-C2 orientation. Preoperative Japenese orthopaedics association score was worst in the lateral tilt and the lateral translation. Correction in all planes was achieved in all patients. CONCLUSIONS: The objective assessment of C1-C2 dislocation and joints in each plane was to determine its management and help in achieving multiplanar correction.

Acute transverse myelitis in childhood: A single centre experience from North India.

BACKGROUND: Acute transvers myelitis (ATM) is a rare and disabling condition in childhood. There are only few reports of clinical profile, prognosis and predictors of ATM from developing countries. OBJECTIVE: To study the clinical profile of children with ATM and predictors of its outcome. METHOD: Retrospective analysis of children <12 years of age diagnosed with ATM over a period of 6 years from a tertiary care institute. RESULTS: Thirty six children (21 boys, median age-7.5 years) were diagnosed with ATM. Weakness was symmetrical at onset in 27 (75%) children with progression over a median of 2 days (IQR 1-5 days). Severe weakness at onset with lower limb power ≤ 1/5 on MRC scale was present in 27 (75%), a sensory level in 25(69.4%) and bladder dysfunction in 31(86.1%) children. MRI showed longitudinal extensive myelitis (LETM) in 27 (75%) children and the thoracic cord was most commonly affected [18 (50%)]. On a median follow up of 35 months (range IQR 11-57 months); 15 (41.7%) were non ambulatory or required assistance to walk. Severe weakness at onset with power ≤ 1 on MRC scale, spinal shock, respiratory muscle weakness, mechanical ventilation, greater mean time to diagnosis and treatment was associated with bad outcome. ATM was a monophasic illness in all, except in 3 children; all with neuromyelitis optica spectrum disorder. Progression to multiple sclerosis was not seen in any child in our cohort. CONCLUSION: In this series of childhood ATM from North India, the disease was severe, monophasic and involved long segments (≥ 3) of cord in majority. Nearly half the children remain dependent on follow up. Delayed diagnosis and delayed initiation of steroid therapy was associated with poor outcome.

Comprehensive drilling of the C1-2 facets to achieve direct posterior reduction in irreducible atlantoaxial dislocation.

OBJECT: The cause of irreducibility in irreducible atlantoaxial dislocation (AAD) appears to be the orientation of the C1-2 facets. The current management strategies for irreducible AAD are directed at removing the cause of irreducibility followed by fusion, rather than transoral decompression and posterior fusion. The technique described in this paper addresses C1-2 facet mobilization by facetectomies to aid intraoperative manipulation. METHODS: Using this technique, reduction was achieved in 19 patients with congenital irreducible AAD treated between January 2011 and December 2013. The C1-2 joints were studied preoperatively, and particular attention was paid to the facet orientation. Intraoperatively, oblique C1-2 joints were opened widely, and extensive drilling of the facets was performed to make them close to flat and parallel to each other, converting an irreducible AAD to a reducible one. Anomalous vertebral arteries (VAs) were addressed appropriately. Further reduction was then achieved after vertical distraction and joint manipulation. RESULTS: Adequate facet drilling was achieved in all but 2 patients, due to VA injury in 1 patient and an acute sagittal angle operated on 2 years previously in the other patient. Complete reduction could be achieved in 17 patients and partial in the remaining 2. All patients showed clinical improvement. Two patients showed partial redislocation due to graft subsidence. The fusion rates were excellent. CONCLUSIONS: Comprehensive drilling of the C1-2 facets appears to be a logical and effective technique for achieving direct posterior reduction in irreducible AAD. The extensive drilling makes large surfaces raw, increasing fusion rates.

Stroke in a case of neonatal lupus: an uncommon complication.

Stroke is an extremely rare complication of congenital heart block in children. We report a 2-year-old girl with congenital complete heart block who presented with acute-onset right middle cerebral artery territory stroke. The congenital heart block was secondary to maternal lupus.

Congenital atlantoaxial dislocation: a dynamic process and role of facets in irreducibility.

OBJECT: Patient age at presentation with congenital atlantoaxial dislocation (CAAD) is variable. In addition, the factors determining irreducibility or reducibility in these patients remain unclear. The facets appear to contribute to the stability of the joint, albeit to an unknown extent. The objective of this paper was to study the characteristics of C1-2 facets in these patients and their bearing on the clinicoradiological presentation and management. METHODS: Twenty-four patients with CAAD were studied. Fifteen patients had irreducible CAAD (IrAAD); 3 of these patients experienced incomplete reduction after traction, and 9 had reducible CAAD (RAAD). The images (CT scans of the craniovertebral junction in a neutral position) obtained in the parasagittal, axial, and coronal planes were studied with respect to the C1-2 facets and were compared with 32 control scans. The inferior sagittal and coronal C-1 facet angles were measured. The lordosis of the cervical spine (cervical spine angle calculated on radiographs of the cervical spine, neutral view) in these patients was compared with normal. The management of these patients is described. RESULTS: The inferior sagittal C-1 facet angle and at least one coronal angle in patients with IrAAD were significantly acute compared with those in patients with RAAD and the control population. A significant correlation was found between age and the acuteness of the inferior sagittal C-1 facet angle (that is, the more acute the angle, the earlier the presentation). The lordosis of the cervical spine was exaggerated in patients with IrAAD. Three patients with IrAAD who had smaller acute angles experienced a partial reduction after traction and a complete reduction after intraoperative distraction of the facets, thereby avoiding a transoral procedure. An inferior sagittal C-1 facet angle of more than 150° in the sagittal plane predicted reducibility. Drilling a wedge off the facet in the sagittal plane to make the inferior sagittal C-1 facet angle 150° can reduce the C1-2 joint intraoperatively by posterior approach alone. CONCLUSIONS: The acuteness of the inferior C-1 sagittal facet angles possibly determines the age at presentation and reducibility. The coronal angles determine the telescoping of C-2 within C-1. Patients with IrAAD can be treated using a posterior approach alone with the exception of those with extremely acute angles or a retroflexed dens. The exaggerated lordosis of the cervical spine in these patients is a compensatory phenomenon.

Acute febrile encephalopathy in adults from Northwest India.

BACKGROUND: Acute onset fever with altered mentation is a common problem encountered by the physician practicing in tropical countries. Central nervous system (CNS) infections are the most common cause resulting in fever with altered mentation in children. AIM: In this study, we have tried to analyze the cause of encephalopathy following short febrile illness in adults presenting to a tertiary care center in Northwestern part of India. SETTING AND DESIGN: A prospective observational study carried out in a tertiary care center in the Northwestern India over a period of 1 year. MATERIAL AND METHODS: A total of 127 patients with fever of less than 2 weeks duration along with alteration in mentation were studied prospectively over a period of 12 months. The demographic variables were recorded in detail. In addition to routine investigations, cerebrospinal fluid analysis, noncontrast- and contrast-enhanced computed tomography, along with magnetic resonance imaging were performed in all the subjects. STATISTICAL ANALYSIS: The results were analyzed using SPSS statistical software. The values were expressed as mean with standard deviation for contiguous variable as percentage for the others. RESULTS AND CONCLUSION: Out of these, 70% had primary CNS infection as the etiology. A total of 33% patients had meningitis, 29.9% had evidence of meningoencephalitis, and 12.7% were diagnosed as sepsis-associated encephalopathy. These were followed by cerebral malaria, leptospirosis, and brain abscess as the cause of febrile encephalopathy in adults. Among the noninfectious causes, acute disseminated encephalomyelitis, cortical venous thrombosis, and neuroleptic malignant syndrome were documented in 2.36% each. In 11% of the patients, the final diagnosis could not be made in spite of the extensive investigations. Our study demonstrates that acute febrile encephalopathy in adults is a heterogeneous syndrome with primary CNS infections being the commonest etiology.

New parameter of hemoglobin status as an indicator of efficacy of preoperative angioembolization in extracranial hypervascular tumours.

BACKGROUND: Routinely, the standard for measuring the success of preoperative embolization procedure as an adjunct in the management of head and neck vascular tumours has been to evaluate the amount of blood loss, duration of surgery, and intraoperative neurovascular injuries. OBJECTIVE: We hypothesized that the rate of change in the preoperative hemoglobin status would more accurately and objectively reflect the effectiveness of the embolization technique. MATERIALS AND METHODS: Twenty-six patients with extracranial vascular tumours were divided into two groups (A and B) of 13. Group A patients had preoperative embolization and group B patients directly underwent surgery. The difference between the preoperative and postoperative hemoglobin levels and the percentage rate of change of hemoglobin status were calculated. RESULTS: The percentage rate of change of preoperative to postoperative hemoglobin is less in group A (9.43%) when compared with group B (18.27%). The ratio of preoperative to postoperative hemoglobin in the two groups is also statistically significant (1:1.9). CONCLUSIONS: The percentage rate of change of preoperative to postoperative hemoglobin and the ratio of preoperative to postoperative hemoglobin are more accurate and objective parameters for assessment of success of preoperative embolizations rather than other variables such as intraoperative blood loss or duration of surgery.