Intraoperative mapping of sacral nervous system (S2-4)

Electrophysiological mapping of the sacral nervous system was used during operations on 80 patients with conus and cauda equina lesions. At surgery, under controlled muscle relaxation, the sacral neural elements (S2-4) were mapped using direct mono-polar stimulation and recording of compound muscle action potentials (CMAPs) from the external anal sphincter (EAS). Responses were obtained in 86.25% (69/80) of the patients. In 33 (82.5%) out of 40 patients with preoperative deficits involving the S2-4 segments, CMAPs could be elicited. Identification of nerve roots was useful in dissection of lipomyelomeningocoeles, tumour excisions and untethering of filum terminale. In three patients, stimulation of the filum terminale elicited motor responses and, hence, it was not sectioned. Intraoperative mapping of the S2-4 nerve roots under controlled muscle relaxation is feasible in a majority of patients, including those with deficits involving S2-4. This method was useful in sparing viable nerve roots during surgery in conus and cauda equina regions, and identification of 'functional' filum terminale.     

Galactorrhea as the sole presenting symptom of a posterior third ventricular epidermoid cyst

BACKGROUND: Brain tumors that are remote from the sellar and parasellar region rarely produce galactorrhea. CASE DESCRIPTION: Galactorrhea was the sole presenting symptom in a patient with a posterior third ventricular epidermoid cyst. On investigation, her serum prolactin level was mildly elevated. After radical excision of the tumour, the spontaneous galactorrhea stopped and the serum prolactin level decreased. CONCLUSION: Because of the clinical and biochemical response seen after surgery, we speculate that the galactorrhea was caused by the presence of the tumor. We postulate that the pressure exerted by the tumor on the diencephalic structures was probably the cause of galactorrhea in our patient.     

Long-term outcome in patients with central neurocytoma following stereotactic biopsy and radiation therapy

Total excision is usually the recommended treatment for central neurocytomas. The role of radiation therapy in their management is controversial, and is usually reserved for partially resected or recurrent tumours. Long-term outcome was studied in patients with central neurocytoma who were treated with radiation therapy. By retrospective chart review, eight patients with intraventricular tumours, who had undergone radiation therapy following stereotactic biopsy, were identified. The diagnosis of neurocytoma was confirmed based on positive immunostaining with synaptophysin. Clinical and radiological follow-up was obtained. Of eight patients, one died 5 years after treatment from shunt dysfunction and 1 had disseminated intracranial disease 15 months after treatment. Six patients were symptom-free at a mean follow-up period of 78 months and had good local control as demonstrated by CT. Since this tumour can be confused with an oligodendroglioma or an ependymoma on routine histopathology (as happened in seven of our cases); immunohistochemical studies with synaptophysin should be routinely performed for intraventricular glial tumours. Our study supports the beneficial effect of radiation therapy in the management of these tumours.     

Unusual MR presentation of cerebral parenchymal tuberculosis

The radiological abnormalities reported in CNS tuberculosis and their pathological correlates are discussed. Focal tuberculous involvement of the CNS without formation of tuberculoma is rare. The MR features in this case were also distinctly unusual for CNS tuberculosis. Therefore, histological confirmation of all lesions thought to be a low grade glioma is mandatory.     

High cervical and lumbar canal stenosis of varied etiology: a case report

Developmental stenosis without any significant spondylotic changes frequently occurs at C3 vertebra or below, and typically extends to C6-C7. However, high cervical focal canal stenosis is unusual. A case of cervical canal segmental stenosis at C2-3 level in addition to a developmental stenosis of the lumbar region, in a 45 year old male, has been presented in this article. The dynamics of the spinal canal in relation to the likely pathology of such conditions are reviewed. We speculate that focal segmental stenosis in the high cervical region may be due to a possible premature fusion of the neurocentral synchondrosis of the cartilage, or due to an abnormal rotary biomechanics which can result in facetal hypertrophy.     

Benign metastasizing leiomyoma causing spinal cord compression

BACKGROUND: Leiomyoma as a cause of cord compression is extremely rare. To our knowledge this is the first report of a dural-based leiomyoma and second report of a leiomyoma causing cord compression. CASE DESCRIPTION: A 38-year-old female renal transplant recipient presented with features of cervical cord compression. On imaging and at surgery, the tumor was mistaken for a neurofibroma. A cervical laminectomy and near total excision of the tumor was done. She did well in the postoperative period but presented 5 months later with thoracic empyema leading to septicemia and her demise. Ultrasound examination and autopsy showed leiomyomas in many other sites including the uterus. CONCLUSIONS: The presence of extrauterine and uterine leiomyomas is a curious condition termed as "benign metastasizing leiomyoma." As our patient was on immunosuppressant therapy following a renal transplant, it might have predisposed her to this rare condition with multifocal tumors.     

Non-traumatic cerebrospinal fluid rhinorrhea: The “Normal pressure leaks”

Summary Non-traumatic cerebrospinal fluid (CSF) rhinorrhea is an uncommon condition. 15 patients with non-traumatic CSF rhinorrhea having normal pressure leaks are presented. Metrizamide CT cisternography failed to reveal the site of the fistula in 3 patients. 11 of the 15 patients underwent surgery for closure of the fistulous communication. In 8 of them the fistula was in the anterior fossa, in one it was in the sellar floor and in two the fistula site was not seen either radiologically or at surgery. In all except the one patient with sellar floor defect an intracranial approach was used. 12 patients have been followed up for 6 months to 9 years and 10 (83%) have been cured of their rhinorrhea. The intracranial approach is preferred to an extracranial approach in the repair of anterior fossa fistulae. Conservative treatment may be effective in selected patients.Presented in part at the 34th Annual Conference of the Neurological Society of India held at Patna from 15–17 December 1985.     

Newer Therapies for Amyloid Cardiomyopathy

The heart and the kidneys are the most commonly involved organs in systemic amyloidosis. Cardiac involvement is associated with an increased morbidity, treatment intolerance, and poorer overall survival. The most common types of amyloidosis that are associated with cardiac involvement include light chain (AL) amyloidosis and transthyretin (TTR) amyloidosis (both mutant and wild type). The traditional first-line treatment for AL amyloidosis includes alkylator-based chemotherapy or high-dose melphalan followed by autologous stem cell transplantation (ASCT). Novel agents, including proteasome inhibitors, immunomodulators, and monoclonal antibodies, have shown promising activity in both frontline and relapsed settings. Orthotopic heart transplantation (OHT) followed by ASCT has led to superior outcomes compared to OHT alone. Orthotopic liver transplantation (OLT) is the first-line treatment for TTR amyloidosis. However, progression of cardiac amyloidosis after OLT is often noted due to deposition of wild TTR. Combined OLT and OHT also has a role in treatment and leads to superior outcomes in carefully selected candidates. Pharmacologic agents, including diflunisal, tafamidis, small interfering ribonucleic acid, and doxycycline, have shown promising activity in stabilizing TTR from misfolding into fibrils and are being actively investigated. Best supportive care and management of heart failure symptoms with diuretics are a mainstay of treatment in all cardiac amyloidosis subtypes. Robust data on the benefit of angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, or beta blockers in amyloid cardiomyopathy is lacking.     

Real-world data on safety and efficacy of venetoclax-based regimens in relapsed/refractory t(11;14) multiple myeloma

The treatment for relapsed/refractory multiple myeloma (RRMM) continues to be challenging despite recent therapeutic advancements. Venetoclax, an inhibitor of the anti-apoptotic protein BCL-2, is a promising agent, especially in patients harbouring t(11;14). Our objective was to review our experience with venetoclax-based regimens at our institution. All ten RRMM patients treated with venetoclax were included and had a median of six prior lines of therapy. The overall response rate was 78% and one patient with cardiac amyloidosis and MM achieved a cardiac organ response. Haematologic toxicities requiring red blood cell and platelet transfusion and non-haematologic toxicities, most commonly gastrointestinal upset, were observed.