Primary intracranial synovial sarcoma: A case report and review of literature.

Background: Primary intracranial synovial sarcomas (PrISS) are unusual dural based mesenchymal tumors seen most commonly in the supratentorial compartment. They can mimic a spontaneous intracranial hemorrhage or a high-grade glioma on imaging. Case Description: A 31-year-old male presented with headache and right hemiparesis for 2 weeks. CT brain revealed a left frontal spontaneous intracerebral hemorrhage. PrISS revealed a heterogeneously ring enhancing solid cystic lesion with attachment to convexity dura. Intraoperatively, it mimicked a high-grade glioma. Histopathology report showed features of a synovial sarcoma, which was later confirmed with IHC. Classical SYT-SSX2 translocation was confirmed only on RTPCR after fluorescent in situ hybridization (FISH) was negative for same. Whole body positron emission tomography (PET-CT) did not show any extracranial tumor. Despite radiotherapy, there were recurrence and tumor progression at 6 months and the patient succumbed 11 months later. Conclusion: PrISS is an unusual aggressive intracranial neoplasm that carries a worse prognosis when compared nonintracranial synovial sarcomas. Molecular cytogenetics (FISH and RTPCR) are essential for confirming the diagnosis, though FISH seems to have a lower sensitivity and can yield false negative results as was noted in this case.

The chlorophenoxy herbicide MCPA: a mechanistic basis for the observed differences in toxicological profile in humans and rats versus dogs.

Metabolism data for MCPA in rat, dog and human shows a single oral dose is quantitatively and rapidly absorbed with evidence of non-linear kinetics at >100 mg/kg bw. The extent of metabolism is low and consistent between rat and human, with substantially higher metabolic conversion in dog. Parent accounts for 50%-67% dose in rat, ∼40% in human and 2%-27% in dog. No dog specific metabolite is apparent.In rat and human, MCPA and metabolites are rapidly eliminated in urine (65%-70% within 24 h) but in dog, excretion is via urine and faeces (20%-30% within 24 h), with renal excretion saturating between 5 and 100 mg/kg bw.The species difference in excretion is reflected in pharmacokinetics. Terminal half-life is similar in rat and human (15-17 h) but higher in dog (47 h). Modelling shows species differences in single dose kinetics profoundly affect systemic exposure following repeat dosing.The difference in renal excretion and systemic exposure of MCPA between dogs and rats has been attributed to species differences in active transporters (OAT1/OAT3). A new in vitro flux study in renal proximal tubules supports this hypothesis with net secretion in rat and human of a similar magnitude but significantly less in dog.

Characterizing syringomyelia and its clinical significance in 140 patients with lipomyelomeningocele.

OBJECTIVE: The aims of this study were to characterize syringomyelia in patients with lipomyelomeningocele (LMMC) and investigate the association between the presence of a syrinx and patient neurological deficits. METHODS: Patients with LMMC who had been admitted between 2007 and 2020 were included in the study. Syringomyelia on magnetic resonance imaging (MRI) was classified into three groups: true syrinx, early syrinx, and presyrinx. The correlation of clinical deficits (at birth, new onset, and progressive) and type of lipoma with the presence and type of syrinx was studied. RESULTS: Among a total of 140 patients, the median age was 2.5 (IQR 1.3-9) years. Neurological deficits were present at birth in 75 (53.6%) patients, but 67 (47.9%) had new-onset deficits or progression of their birth deficits. The most common type of spinal lipoma was the transitional type (75.7%). Thirty-nine (27.9%) patients had a syrinx (37 with a true syrinx, 2 with an early syrinx), and 25 (17.9%) patients had a presyrinx. There was no significant correlation between the presence of a syrinx and patient neurological deficits. However, patients with a syrinx that was not adjacent to the lipoma (≥ 1 vertebral level away) had a higher incidence of deficits at birth (p = 0.045), whereas those with a syrinx spanning ≥ 5 vertebral levels had a higher incidence of progressive neurological deficits (p = 0.04). Six (75%) of 8 patients in whom serial MRI had shown syrinx progression had clinical worsening. CONCLUSIONS: Syrinx location with respect to the spinal lipoma, syrinx length, and progressive syringomyelia may have clinical significance in patients with LMMC.

Risk factors for pre-operative functional deterioration in children with lipomyelomeningocele.

PURPOSE: To characterize the temporal profile of pre-operative deterioration in children with lipomyelomeningocele (LMMC) including those with congenital deficits and identify risk factors for clinical worsening. METHODS: Records of 87 children who underwent surgery for LMMC were retrospectively reviewed to study the temporal profile of pre-operative deterioration, defined by the onset of new neurological dysfunction or progression of a pre-existing deficit. Preoperative magnetic resonance imaging (MRI) studies were examined to identify radiological features associated with deterioration. In children with extra-spinal placodes, the angle subtended by the terminal placode with the cord at the level of the laminar-fascial defect ("J sign") was assessed. RESULTS: Pre-operative deterioration in function was seen in 37 children (43%), occurring at a median age of 36 months and was more frequent in children without congenital deficits (54% versus 27%; p = 0.016). On Cox regression analysis, extra-spinal location of the placode (p = 0.003) and presence of a congenital deficit (p = 0.009) were positively and negatively associated with deterioration respectively. On Kaplan-Meier analysis, the median deterioration-free survival time was 72 months and was positively associated with presence of congenital deficit (p = 0.026) and negatively associated with presence of an extra-spinal placode (p < 0.001) or a meningocele sac (p = 0.001). CONCLUSION: Extra-spinal location of the neural placode was associated with higher risk of clinical deterioration in children with LMMC, whereas the presence of a congenital deficit conferred a decreased risk. Risk stratification based on clinical and radiological features can be used to guide decisions regarding early prophylactic surgery in children with LMMC.

Early- and long-term surgical outcomes in 109 children with lipomyelomeningocele.

AIM: To determine the functional outcomes and risk factors for outcomes following surgery for lipomyelomeningocele (LMMC). METHODS: Data from 109 children with LMMC who underwent surgery from January 2008 to December 2017 were retrospectively studied to evaluate functional outcomes and possible risk factors for early- (at discharge from hospital or within 1 month of surgery) and long-term outcomes after surgery. RESULTS: There were 53 boys and 56 girls with median age of 36 months (IQR 12-90 months; range, 4 months to 18 years) at surgery. At presentation, neurological function was normal (asymptomatic group) in 28 (25.7%) children while there was neurological dysfunction (symptomatic group) in 81 (74.3%). Near total or radical excision of lipoma was done in 71 (65.1%) children. Six (5.8%) children had deterioration of neurological function in the post-operative period with recovery of function in three of them. There were no statistically significant risk factors for early outcomes. At a mean follow-up of 62.5 months (IQR 35-82 months; range, 12-146 months), 21 (27.6%) of the 76 symptomatic group children (including 59.3% with incomplete bladder dysfunction) showed improvement, 52 (68.4%) remained the same, while 3 (4%) deteriorated. In 31 children (symptomatic group) who developed symptoms after 1 year of age, early surgery after development of symptoms was associated with better chances of recovery (p = 0.0008). In the asymptomatic group, 23 (88.5%) had normal neurological function at mean follow-up of 57.5 months (IQR 30-77 months; range, 12-141 months) and 3 (11.5%) had neurological deterioration. The mean time to late deterioration (re-tethering) from surgery was 51.3 months (IQR 24-75 months; range, 24-84 months). Presence of syrinx on initial MRI (p = 0.008) and partial resection of the lipoma (p = 0.02) were independent risk factors for delayed deterioration. CONCLUSION: Radical resection of LMMC probably helps in preserving neurological function in > 90% of children at long-term follow-up. Fifty-five percent of children > 2 years of age with incomplete bladder dysfunction regained normal function following surgery. In children who develop symptoms after birth, early surgical intervention can reverse the neurological deficits.

Craniovertebral Junction Arachnoiditis: An Unusual Sequelae to Tuberculous Meningitis.

Adhesive arachnoiditis at the craniovertebral junction should be suspected in patients with a history of meningitis having delayed onset gradually progressive tetra paresis. Patients can present after an asymptomatic interval of 2 to 20 years. Cardiac gated cine magnetic resonance imaging is useful for its diagnosis. Posterior fossa decompression with upper cervical laminectomy and adhesiolysis appears to be a reasonable treatment for the same. We illustrate two patients who presented to us with gradually progressive spastic tetra paresis; both had prior history of cured tuberculous meningitis.

Intracranial pressure monitoring in diffuse brain injury-why the developing world needs it more?

BACKGROUND: Use of ICP monitoring is considered to be part of "standard of care" in management of severe traumatic brain injury, but it is rarely used in developing countries. The authors present a study which evaluates the efficacy and outcomes of ICP monitoring at a high-volume trauma center in India. METHODS: Data on management and outcomes for 126 patients who were admitted with diffuse traumatic brain injury (GCS 3-8) were studied prospectively over an 18-month period. These patients were treated by one of the two specific protocols: ICP monitoring-based or non-ICP monitoring-based. The primary outcome was measured based on 2 weeks mortality and GOS-E at 1, 3, and 6 months. Secondary outcome was measured based on need for brain-specific treatment, length of ICU stay, and radiation exposure. RESULTS: Mortality in a subset of patients who underwent surgical intervention later due to increased ICP values, drop in GCS, or radiological deterioration was noted to be significantly lower in the ICP monitoring group (p = 0.03), in spite of statistically insignificant difference in overall mortality rates between groups. GOS-E scores at 1 month were significantly better (p = 0.033) in ICP monitoring group, even though they equalized at 3 and 6 months. The need for brain-specific treatment (p < 0.001), radiation exposure (p < 0.001), and length of ICU stay (p = 0.013) was significantly lower in the ICP monitoring group. CONCLUSIONS: ICP monitoring-based treatment protocol helps in achieving faster recovery; lowers mortality rates in operated patients; and reduces ICU stay, radiation exposure, and the need for brain-specific treatment.

Traumatic Chiasmal Syndrome: A Midline Axonal Injury.

We report a 26-year-old man who developed cerebrospinal fluid (CSF) rhinorrhoea and complete bitemporal hemianopia after having sustained a blunt frontal region trauma. The chronology of midline frontal trauma and CSF rhinorrhoea, followed days later by transient diabetes insipidus and discovery of a bitemporal visual field loss, makes up a unique syndrome and must be evaluated for. Mechanism of such injuries, their pertinent complications, and review of literature are herein discussed.

Rare case of bilateral vertebral artery stenosis caused by C4-5 spondylotic changes manifesting with bilateral bow hunter's syndrome.

BACKGROUND: Rotational vertebral artery occlusion syndrome refers to vertebrobasilar insufficiency as a result of mechanical occlusion or stenosis of the vertebral artery by head rotation. In most cases, symptoms are produced on extension or rotation to one side. No case of bow hunter's syndrome with bilateral presentation at the C4 level has yet been reported. CASE DESCRIPTION: A 54-year-old man presented with symptomatic bilateral bow hunter's syndrome induced by head rotation. The patient complained of intermittent dizziness, episodes of double vision, nonpulsatile tinnitus, and headaches indicative of vertebral artery insufficiency with exacerbation of symptoms on rotation of his head to either side. Computed tomography angiography showed bilateral vertebral artery stenosis, and dynamic cerebral angiography revealed bilateral rotational vertebral artery occlusion, with compression of the ipsilateral vertebral artery on head rotation to either side. Bilateral surgical decompression at C4-5 with anterior cervical diskectomy and fusion with a plate was performed. CONCLUSIONS: Bony obstruction of the vertebral artery on head rotation tends to occur at levels C4 and below, affecting the ipsilateral side. In this rare case, symptomatic bilateral vertebral artery stenosis occurred as a result of bony compression and was symptomatic on head rotation both to the right and to the left. This stenosis was improved with anterior decompression bilaterally, and no further events occurred postoperatively.