Venous pathology targeted surgical management in Hirayama disease: A comprehensive case series of nine cases exploring this potential etiology.

Objective: Hirayama disease is a rare cause of cervical myelopathy predominantly affecting young individuals. The disease is classically characterized by muscle atrophy in the distal upper limbs. While various etiopathogenesis such as dural sac dysplasia, nerve root dysplasia, structural abnormalities of the spinal ligament, and venous dysplasia have been proposed, this study explores the potential role of venous pathology and surgical management on the basis of it. Methodology: This is a prospective descriptive case series of nine cases. The diagnosis was made based on the Huashan diagnostic criteria which includes clinical manifestation, imaging, and electrophysiology. In cases where magnetic resonance imaging (MRI) failed to demonstrate engorged veins, a computed tomography (CT) venogram of the cervical spine was used as an imaging tool. All patients underwent cervical laminectomy and coagulation of the posterior epidural venous plexus with or without laminoplasty. All the patients were followed up regularly; clinical improvement and neck disability index were assessed. Results: All nine patients were male and exhibited classical clinical features, electrophysiological abnormalities, and MRI findings except, in one patient where a CT venogram helped in establishing the diagnosis as the MRI was inconclusive. Postoperatively, all patients had neurological improvement and stabilization of the disease. All patients who underwent CT venogram and cervical spine X-ray in neutral and dynamic position demonstrated no recurrence of engorged venous plexus or significant instability except one patient developing kyphosis. One patient experiencing symptoms in the other limb underwent a second surgery. Conclusion: This comprehensive case series strongly supports venous pathology as a potential etiology of Hirayama disease. Surgical management with laminectomy and venous coagulation with or without expansile laminoplasty has delivered consistent improvement in neurological outcomes and long-term disease stabilization without the restriction of movements and lesser complications. However, further research is warranted to elucidate the mechanism underlying cervical venous dilatation.

Radiosurgery for Trigeminal Neuralgia Secondary to Dolichoectatic Vessels: Case Series and Review of Literature.

Even for seasoned neurosurgeons who have mastered the microvascular decompression (MVD) techniques, trigeminal neuralgia (TGN) secondary to vertebrobasilar dolichoectatic vessels remains a challenge. Often, patient is either medically infirm or unwilling for invasive surgical interventions. Alternative treatment options including Gamma Knife radiosurgery (GKRS) are considered in such a situation with variable success. This study aimed to evaluate the role of GKRS in patients with trigeminal neuralgia with dolichoectatic vessels and severe cross compression. We prospectively managed three male patients of intractable TGN secondary to dolichoectatic vascular compression with single-session GKRS. The cisternal component of the trigeminal nerve was targeted with 90 Gy radiation at 100% isodose with a single 4-mm collimator. The patients were regularly evaluated on clinical parameters for pain relief (Barrow Neurological Institute (BNI) score), sensory complaints, and outcome. All patients had satisfactory pain control (BNI I-IIIa) at 3 months of interval only to get recurrent pain (BNI IV-V) after 6 months. The treatment was eventually considered a failure after 6-month duration and one patient needed MVD for pain control. Post-GKRS pain control remains inferior in patients with dolichoectasia compared with typical TGN. GKRS should be offered only as a salvage or rescue procedure and should not be used as an alternative treatment of MVD in patient population with dolichoectatic vessels.

Radiosurgical Corpus Callosotomy: A Review of Literature.

BACKGROUND: Microsurgical callosotomy is a procedure still under debate and to best a palliative treatment for drug-resistant epilepsy. Unlike microsurgery, radiosurgical callosotomy is an underpracticed treatment option, with no definite account of its safety and outcome profile. OBJECTIVE: To evaluate the safety, efficacy, and complication profile of radiosurgical callosotomy in the literature. METHODS: PubMed, SCOPUS, Web of Science, and ResearchGate were reviewed for radiosurgery and callosotomy in the English language following PRISMA guidelines. The patient profile, radiosurgical parameters (dose and isodose), target volume, extent of radiosurgery (anterior third, half, or posterior third callosotomy), and seizure outcome were evaluated. We evaluated the role of radiosurgery as a primary or secondary treatment modality after microsurgery. A literature review was performed to identify the evidence of radiosurgery. RESULTS: We identified 7 studies detailing 12 patients of mean age 22.8 years (range, 4-58 years) and a mean of 18.9 years of illness (range, 5-37 years). Five series performed Gamma Knife radiosurgery and 2 performed LINAC radiosurgery. The spectrum of seizures ranged from atonic seizures/drop attack (83%), generalized tonic-clonic seizures (75%), complex partial seizures (67%), absence seizures (50%), myoclonic seizures (33%), to focal seizures (16%). Four patients suffered from Lennox-Gastaut syndrome. The average seizure frequency in 11 patients was 297/month (range, 20/day to 15/month). Three patients became free of drop attacks and 2 free of generalized tonic-clonic seizures, and 1 became completely seizure free. The remaining patients continued to have seizures, albeit at a lower frequency. Complex partial seizures and myoclonic seizures were the least responsive seizure types to radiosurgical corpus callosotomy. All patients tolerated the procedure well. After radiosurgery, 3 patients developed symptomatic edema. The symptoms (headache, nausea, hemiparesis, and transient neurologic deficits) were controlled with a short course of steroids. Two patients needed redo radiosurgery (at the same target in 1 patient and complementary middle third callosotomy to previous anterior third callosotomy in another patient). There were no long-term complications. CONCLUSIONS: Radiosurgery is a viable alternative to microsurgical callosotomy both as a primary and as a secondary treatment modality. It has a specific advantage of better neuropsychological outcomes with comparable seizure control. The neurosurgical community should adopt a more liberal approach with this indication.

An unusual presentation of neurofibroma masquerading as a vascular hamartoma, post-iatrogenic intervention.

Neurofibroma is a localized discrete mass of benign nerve sheath tumour in the peripheral nervous system. Mostly present as skin lesions. Solitary neurofibroma may occur in deep soft tissue or subcutaneous plane in rare cases associated with syndromes like NF1. The neurofibroma most commonly present as skin lesions as isolated soft papules or nodules arising in any cutaneous site. Present case depicts unusual presentation of a neurofibroma as a vascular hamartoma post an iatrogenic intervention.