Goutham Cugati

Primary spinal epidural lymphomas

An epidural location for lymphoma is observed in 0.1–6.5% of all the lymphomas. Primary spinal epidural lymphoma (PSEL) is a subset of lymphomas, where there are no other recognizable sites of lymphomas at the time of diagnosis. The incidence of this subset of lymphomas is much less. It, however, is increasingly diagnosed, due to the increased use of more sensitive imaging modalities. For the electronic search, Pubmed was used to identify journals that enlisted and enumerated PSEL from 1961 to January 2011. The following combination of terms: “primary,” “spinal,” “epidural,” and “lymphoma” were used. The most significant articles and their bibliographies were analyzed by the authors. The symptoms, pathogenesis, diagnostic workup, histopathology, treatment, and outcome have been analyzed in a systematic manner

Hyperprolactinemia: An often missed cause of male infertility.

1. Tu HY, Zini A. Finasteride-induced secondary infertility associated with sperm DNA damage. Fertil Steril 2011;95:2125.e13-4. 2. Liu KE, Binsaleh S, Lo KC, Jarvi K. Propecia-induced spermatogenic failure: A report of two cases. Fertil Steril 2008;90:849.e17-9. 3. Chiba K, Yamaguchi K, Li F, Ando M, Fujisawa M. Finasteride-associated male infertility. Fertil Steril 2011;95:1786.e9-11. 4. Amory JK, Wang C, Swerdloff RS, Anawalt BD, Matsumoto AM, Bremner WJ, et al. The effect of 5alpha-reductase inhibition with dutasteride and finasteride on semen parameters and serum hormones in healthy men. J Clin Endocrinol Metab 2007;92: 1659-65. 5. Parivar K, Yaghmaei P, Sekhavati S. The role and effect of finasteride on the spermatogenesis, prostate gland and epididym of mature NMRI mouse in in vivo and in vitro conditions. Med Sci J Islam Azad Univ 2008;179:2333-8. 6. Laborde E, Brannigan RE. Effect of 1-mg dose of finasteride on spermatogenesis and pregnancy. J Androl 2010;31: e1-2. 7. Overstreet JW, Fuh VL, Gould J, Howards SS, Lieber MM, Hellstrom W, et al. Chronic treatment with finasteride daily does not affect spermatogenesis or semen production in young men. J Urol 1999;162:1295-300.

Multiple burr hole surgery as a treatment modality for pediatric moyamoya disease.

Objective: To re-emphasize that indirect revascularization surgery alone, where multiple burr holes and arachnoid openings are made over both cerebral hemispheres, is beneficial in the treatment of moyamoya disease (MMD) in children. Clinical Presentation: We report a 10-year-old boy who presented with complaints of episodic headache for the last 5 years. At the peak of his headache he had visual disturbances and acute onset weakness of left-sided limbs, recovering within a few minutes. He had no focal neurological deficits. Radiological investigations revealed abnormal findings, demonstrating the features of MMD. Surgical Management: He underwent bilateral multiple burr holes, dural and arachnoid opening over the frontal, parietal and temporal regions of each hemisphere. The elevated periosteal flap was placed in contact with the exposed brain through each burr hole. Results: On 6-months follow-up he had only one episode of transient ischemic attack. Postoperative four vessel angiogram demonstrated excellent cerebral revascularization around the burr hole sites, and single photon emission computerized tomography imaging showed hypoperfusion in the right temporo-occipital area suggestive of an old infarct with no other perfusion defect in the rest of the brain parenchyma. Conclusion: In children with MMD this relatively simple surgical technique is effective and safe, and can be used as the only treatment without supplementary revascularization procedures. This procedure can be done in a single stage on both sides and the number of burr holes made over each hemisphere depends on the extent of the disease.

Primary intrasellar schwannoma

Intracranial schwannomas commonly arise from the eighth cranial nerve in the cerebellopontine angle. Schwannoma arising in the sella and extending into the suprasellar region is very rare and is easily mistaken for pituitary adenoma. To our knowledge, there have been only 12 previous reports. We present a patient with primary intrasellar schwannoma that clinically and radiologically resembled a pituitary adenoma (PA). Intra-operative findings differed from a PA, as the tumour had a firmer consistency. Gross total excision of the lesion was done via a transethmosphenoidal approach. Post-operatively the patient improved in visual acuity and visual fields. We have reviewed the literature and described the characteristics of such lesions.

Complication avoidance in transcallosal transforaminal approach to colloid cysts of the anterior third ventriclen: An analysis of 80 cases

Object: The objective of the present study is to analyze the complications and their avoidance in a series of 80 patients operated by transcallosal transforaminal approach to colloid cysts of the anterior third ventricle. Materials and Methods: The surgical outcome and complications of 80 patients operated by transcallosal transforaminal approach for colloid cysts in the anterior third ventricle was analyzed. A detailed pre- and post-operative neurological assessment was done in all patients. Neurocognitive assessment of corpus callosal function was done in the last 22 cases. CT scan of the brain was done in all patients, before and after surgery. Results: All patients underwent transcallosal transforaminal approach. Total excision of the lesion was achieved in 79 patients and subtotal in one. The complications encountered were postoperative seizures in six, acute hydrocephalus in four, venous cortical infarct in four, transient hemiparesis in four, transient memory impairment, especially for immediate recall in nine, mutism in one, subdural hematoma in one, meningitis in three, and tension pneumocephalus in one patient. There were two mortalities. There was no incidence of postoperative disconnection syndrome. Conclusion: Colloid cyst is surgically curable. With good knowledge of the regional anatomy and meticulous microsurgical techniques, there is a low mortality and minimum morbidity, when compared to the natural history of the disease. With increasing experience, most of the complications are avoidable. The limited anterior callosotomy does not result in disconnection syndromes.

Primary spinal intradural extramedullary lymphoma causing cauda equina syndrome.

We report a case of lumbar intradural extramedullary lesion in an 11-year-old boy who presented with cauda equina syndrome and acute bladder disturbance. He underwent emergency surgical resection of the lesion, which was proved to be a lymphoma by histopathology and immunohistochemistry. He has improved neurologically and after 1 year, he is leading a normal life with near normal neurological functions. This is the second case of primary spinal intradural extramedullary lymphoma. This is the first such case in the pediatric age group and causing cauda equina syndrome. We describe the characteristics of such tumors along with pathogenesis and management.

Extra axial adult cerebellopontine angle medulloblastoma: An extremely rare site of tumor with metastasis

Dear Sir, Medulloblastomas are rarely seen in the adult population, accounting to less than 1% of primary adult brain tumors.[6] It commonly arises from the cerebellar vermis.[5,6] There are only a few cases of cerebellopontine (CP) angle medulloblastomas. Most of them are intra-axial. Extra-axial site of this tumor is extremely rare.[2] Ours is the first case of metastasis from CP angle medulloblastoma. A 22-year-old male patient presented with headache, vomiting, ataxia, and left facial weakness of one-month duration. Vision was normal. Fundus examination showed papilloedema. He had left lower motor neuron facial paresis, left IX and X cranial nerve paresis, and left cerebellar signs. Computed tomography scan of the brain showed a left CP angle mixed-density nonenhancing lesion (5.4 × 2.8 cm) with broad-based tentorial attachment and displacement of the fourth ventricle causing obstructive hydrocephalus [Figure 1]. Findings were confirmed by magnetic resonance imaging (MRI) [Figure 2]. He had previously undergone right ventriculo peritoneal shunt for obstructive hydrocephalus elsewhere. He underwent left retromastoid craniectomy and gross total excision of the lesion. There was clear plane between the tumor and cerebellum, whereas it was adherent to tent laterally. Histopathology showed a highly cellular tumor composed of rosettes of small round cells, with high nucleus-cytoplasm ratio and increased mitotic figures—suggestive of classical medulloblastoma—WHO grade IV [Figure 3]. Postoperatively, he improved. He was advised craniospinal radiotherapy which he failed to receive and presented 15 months later with progressive quadriparesis and sensory impairment of five-month duration with bladder and bowel involvement. On examination, he had 3/5 motor power in the upper limbs and 2/5 in lower limbs and sensory impairment below D4 dermatome. All deep tendon reflexes were brisk with bilateral extensor plantar reflexes. All superficial reflexes were absent. MRI of the brain and spine showed recurrent left CP angle medulloblastoma and intramedullary lesion at cervical level and intradural extramedullary lesion at sacral level, suggestive of drop metastasis in the spine from CP angle medulloblastoma [Figure 4]. Figure 1 Contrast Computed tomography scan of the brain axial and coronal reconstruction showed a left CP angle mixed-density nonenhancing lesion with broad-based tentorial attachment Figure 2 MRI of the brain axial T1, T2 and coronal sections showed a left CP angle lesion with broad-based tentorial attachment Figure 3 Microphotograph of the histopathology slide. (H and E, ×20). showed a highly cellular tumor composed of rosettes of small round cells, with high nucleus-cytoplasm ratio —suggestive of medulloblastoma Figure 4 Saggital MRI of the cervical and sacral region showing drop metastasis (shown by arrows) Medulloblastoma usually occur in inferior medullary velum in the midline.[6] However, rarely they may occur laterally in the cerebellar hemisphere in adults.[3] Origin of medulloblastoma may be either from germinal cells or their remnants situated at the end of posterior medullary velum or from remnants of the external granular layer.[5,7] Their development in the CPA may be from the remnants of the external granular layer in the cerebellar hemisphere, including the flocculus which faces the CP angle.[5] In CP angle medulloblastomas, though fifth, sixth, and eighth cranial nerves are frequently involved,[4] these nerves were spared in this patient. CP angle medulloblastomas are rare with nearly 35 cases published in the literature,[2,5] of which only 9 are in adults.[2,5] The lack of association with any cerebellar tissue and extra-axial location in the region of CP angle is an extremely rare phenomenon.[2] Medulloblastomas are known to metastasize through CSF to the spinal canal, leptomeninges, and supratentorial regions. Metastasis in medulloblastomas vary between 38 and 60% in various series,[1,6] with the spinal canal being the commonest site with approximately 58%.[6] Kumar et al. have reported a case of vermian medulloblastoma with metastasis to the CP angle.[5] To the best of authors’ knowledge, spinal metastasis from CP angle medulloblastoma has not been reported till date. Extra-axial site of this tumor is extremely rare but must be considered in the differential diagnosis of extra-axial CP angle lesions. Any neurological deterioration seen in follow-up patient must be evaluated for metastasis.

Fingolimod: The first oral drug approved by food and drug administration; A breakthrough in treatment of multiple sclerosis

Sir, In continuation of our effort to deeply understand the reactions of triazinone, we carried out the optimization of sodium acetate, which is utilized as a catalyst in the reaction mechanism.[1] We carry forward the synthesis of triazinone, which begins with 4-(4-bromobenzylidene)-2-phenyloxazol-5(4H)-one and acid hydrazide. During reaction monitoring, a light spot started appearing on the thin layer chromatography (TLC) after 4 h of the commencement of the reaction, which eventually deepened after 12 h. On cooling, this reaction mixture produced white colored crystals, which were earlier presumed to be triazinone. The fourier transform infrared (FT-IR) spectrum reflected the presence of two sharp stretchings above 3,000 cm-1, but it was expected to be only one. The two sharp stretchings were observed at 3456.39 and 3231.78, which signifies the presence of two acidic protons, i.e., -NH or -OH, while in 1HNMR spectrum, a singlet was expected for –CONH at 11-12 ppm, which was absent in our spectra. Instead, a singlet appeared at 9.6 ppm; apart from this, the presumed number of protons was 18, which was found to be only 12. Also, the total number of singlets were expected to be 2 (-CH, -NH), which were found to be 3, of which 2 were displaceable. This suggested the presence of two acidic protons in the synthesized compound. Further, the mass spectra showed a peak at 368, which was expected to be at 475. On further investigation of 1HNMR and mass fragmentation pattern, it was found that the synthesized compound was not triazinone; rather, it was a hydrolyzed product of 4-(4-bromobenzylidene)-2-phenyloxazol-5(4H)-one, i.e., acrylic acid derivative, having molecular weight 345, which appeared at 368 (with the peak of Na+) in our spectrum. This was further assured by separately carrying out the hydrolysis of 4-(4-bromobenzylidene)-2-phenyloxazol-5(4H)-one in glacial acetic acid [Scheme 1] and comparing its TLC and spectral data with our synthesized compound. This acrylic acid derivative is not yet reported. Kapish Madaan, Tarawanti Verma, Darpan Kaushik, Nancy, Nanjan Mahadevan Department of Pharmaceutical Chemistry, Rajendra Institute of Technology and Sciences, 4th Mile Stone, Hisar Road, Sirsa-125 055, India E-mail: darpkaush@yahoomail.com

Bromocriptine or cabergoline induced pituitary apoplexy: Rare but life-threatening catastrophe

Sir, Prolactin secreting pituitary tumor is one of the common causes of infertility in males and females.[1] Prolactin level is also high in non-prolactin secreting pituitary adenoma (e.g., growth hormone secreting) due to stalk effect.[2] Most of the prolactinomas can be managed medically with bromocriptine or cabergoline which are largely used as primary treatment for prolactinomas, as they help to normalize serum prolactin levels and induce reduction in the tumor size, promoting restoration of gonadal function, cessation of galactorrhea and improvement in visual defects in the majority of patients.[1,2] Bromocriptine or cabergoline induced pituitary apoplexy is a life-threatening complication which is rare but well known.[2-5] This condition is characterized by sudden onset of headache, visual loss or deterioration, meningismus, altered mental status, and rarely, even death.[1,2] This pathology is caused by hemorrhagic necrosis of tumor or pituitary gland infarction, in which pituitary function is compromised, necessitating rapid administration of corticosteroids and endocrine evaluation.[1] Rapid surgical decompression is required if there is sudden constriction of visual fields, and/ or rapid deterioration of acuity, or neurological deterioration due to hydrocephalus.[1,2] These reports point toward the possibility of bromocriptine or cabergoline induced pituitary apoplexy, which should be kept in mind when a patient is neurologically deteriorating after starting these drugs. Careful follow-up is required when treatment with bromocriptine or cabergoline is attempted for prolactinoma in reproductive medicine clinic for infertility management in male and female patients.

Effect of epilepsy on female fertility and reproductive abnormalities.

Journal of Human Reproductive Sciences / Volume 4 / Issue 2 / May Aug 2011 endocrinal disturbances.[2] They may interfere with the hypothalamic pituitary axis and produce amenorrhea, oligomenorrhea, and prolonged and irregular cycles. [2] Higher incidence of PCOD is also reported in women taking valproate sodium, and a retrospective series reported PCOD in 43% of women taking valproate for epilepsy.[2,6] A recent study done in India, concluded that infertility was least common (7.1%) in those epileptic women who were not on antiepileptic drug (AED) exposure compared to those who were with AED exposure (31.8% with one AED exposure, 40.7% with two AED exposures, and 60.3% with three or more AED exposures).[1] Epileptic women who were taking phenobarbital had a significantly higher risk of infertility, but no such trend was observed with other AED drugs.[1] Infertility was also common in epileptic women who were older in age, with lower education.[1]