Showing papers in "Neurology India in 2008"
TL;DR: Patients with DM or PM usually respond to treatment with glucocorticoids and immunosuppressive agents but their use remains largely empirical; emerging therapies for resistant cases include TNFalpha inhibitors (etanercept, infliximab) and monoclonal antibodies (rituximab, alemtuzumab).
Abstract: The three major immune-mediated inflammatory myopathies, dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM), each have their own distinctive clinical features, underlying pathogenetic mechanisms and patterns of muscle gene expression. In DM a complement-dependent humoral process thought to be initiated by antibodies to endothelial cells results in a microangiopathy with secondary ischemic changes in muscles. On the other hand, in PM and IBM there is a T-cell response with invasion of muscle fibers by CD8+ lymphocytes and perforin-mediated cytotoxic necrosis. In IBM degenerative changes are also a feature and comprise autophagia with rimmed vacuole formation and inclusions containing β-amyloid and other proteins whose accumulation may be linked to impaired proteasomal function. The relationship between the inflammatory and degenerative component remains unclear, as does the basis for the selective vulnerability of certain muscles and the resistance to conventional forms of immunotherapy in most cases of IBM. Patients with DM or PM usually respond to treatment with glucocorticoids and immunosuppressive agents but their use remains largely empirical. Intravenous immunoglobulin therapy can be used to achieve disease control in patients with severe weakness or dysphagia, or in patients with immunodeficiency, but its use is limited by expense. Emerging therapies for resistant cases include TNFα inhibitors (etanercept, infliximab) and monoclonal antibodies (rituximab, alemtuzumab). However, experience with these therapies is still limited and there is a need for randomized trials to test their efficacy and establish guidelines for their use in clinical practice.
209 citations
TL;DR: Advances in the management of DMD, including treatment with corticosteroids and the use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder.
Abstract: Duchenne muscular dystrophy (DMD), an X-linked disorder, is the most common muscular dystrophy in children, presenting in early childhood and characterized by proximal muscle weakness and calf hypertrophy in affected boys. Patients usually become wheelchair-bound by the age of 12 years, and die of cardiorespiratory complications in their late teens to early twenties. Advances in the management of DMD, including treatment with corticosteroids and the use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder. The clinical features, investigations, and management of DMD are reviewed, as well as the latest in some of the novel therapies.
74 citations
TL;DR: It is concluded that the described method of atlantoaxial joint distraction and fixation provides an alternative treatment strategy for cases with basilar invagination and joint distraction as a stand-alone method could provide reduction of basilar invasion and firm stabilization in such cases.
Abstract: Objective: A novel method of treatment of basilar invagination that involves distraction of the atlantoaxial joint using specially designed spiked spacers is described Bone graft that is additionally placed within the appropriately prepared atlantoaxial joint and posterior to the arch of atlas and lamina of C2 provides bony fusion Materials and Methods: Between December 2002 and April 2007, 11 patients underwent the discussed method of fixation at the Department of Neurosurgery, King Edward Memorial Hospital in Mumbai, India All 11 patients had congenital basilar invagination and the symptoms were progressive in nature Results: The mean follow-up period was 21 months (range 8-40 months) Neurological improvement and successful distraction with atlantoaxial stabilization and ultimate bone fusion was achieved in all the patients and was documented with dynamic radiography There were no neurological, vascular, or infective complications Conclusions: We conclude that the described method of atlantoaxial joint distraction and fixation provides an alternative treatment strategy for cases with basilar invagination Joint distraction as a stand-alone method could provide reduction of basilar invagination and firm stabilization in such cases
58 citations
TL;DR: The mortality in patients who developed meningitis was high and the high percentage of concurrent nosocomial infections was associated with a high mortality rate which was a serious problem.
Abstract: Background: Nosocomial meningitis is a rare complication following neurosurgical procedures and is associated with high morbidity and mortality. Aim: The aim of this study was to describe the clinical characteristics and the risk factors associated with mortality in patients who developed nosocomial meningitis following neurosurgical operations. Setting and design: Tertiary care hospital and an observational study. Materials and Methods: The study subjects included 2265 patients who underwent various neurosurgical operations during 2003-05. The diagnosis of nosocomial meningitis was based on the Center for Disease Control criteria. Statistical analysis: It was performed by using Statistical Package for Social Sciences for Windows 10.0 program. Results: The incidence of postoperative nosocomial meningitis was 2.7% (62 episodes in 49 patients among 2265 patients operated). Staphylococcus aureus and Acinetobacter spp. were the most frequently isolated pathogens. Of the 49 with meningitis 20 (40.8%) patients died. In the logistic regression analysis model, Glascow coma scale score less than 10 (Odds Ratio (OR): 19.419, 95% Confidence Interval (CI); 1.637-230.41, P = 0.001), and low cerebrospinal fluid glucose level (≤ 30 mg/ dL) (OR: 10.272, 95% CI; 1.273-82.854, P = 0.002), and presence of concurrent nosocomial infection (OR: 28.744, 95% CI;1.647-501.73, P =0.001) were the independent risk factors associated with mortality. Conclusion: The mortality in patients who developed meningitis was high. The high percentage of concurrent nosocomial infections was associated with a high mortality rate which was a serious problem.
52 citations
TL;DR: Gamma knife radiosurgery is a safe and effective primary and secondary modality of treatment for Glomus jugulare tumors.
Abstract: Context: Glomus jugulare (GJ) tumors are paragangliomas found in the region of the jugular foramen. Surgery with/without embolization and conventional radiotherapy has been the traditional management option. Aim: To analyze the efficacy of gamma knife radiosurgery (GKS) as a primary or an adjunctive form of therapy. Settings and Design: A retrospective analysis of patients who received GKS at a tertiary neurosurgical center was performed. Materials and Methods: Of the 1601 patients who underwent GKS from 1997 to 2006, 24 patients with GJ underwent 25 procedures. Results: The average age of the cohort was 46.6 years (range, 22-76 years) and the male to female ratio was 1:2. The most common neurological deficit was IX, X, XI cranial nerve paresis (15/24). Fifteen patients received primary GKS. Mean tumor size was 8.7 cc (range 1.1-17.2 cc). The coverage achieved was 93.1% (range 90-97%) using a mean tumor margin dose of 16.4 Gy (range 12-25 Gy) at a mean isodose of 49.5% (range 45-50%). Thirteen patients (six primary and seven secondary) were available for follow-up at a median interval of 24 months (range seven to 48 months). The average tumor size was 7.9 cc (range 1.1-17.2 cc). Using a mean tumor margin dose of 16.3 Gy (range 12-20 Gy) 93.6% coverage (range 91-97%) was achieved. Six patients improved clinically. A single patient developed transient trigeminal neuralgia. Magnetic resonance imaging follow-up was available for 10 patients; seven recorded a decrease in size. There was no tumor progression. Conclusions: Gamma knife radiosurgery is a safe and effective primary and secondary modality of treatment for GJ.
49 citations
TL;DR: Dynamic post contrast MRI evaluation of cervicothoracic spine is an accurate method for the diagnosis of Hirayama disease.
Abstract: Department of Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India. gupta209@gmail.com.
49 citations
TL;DR: Two cases are reported as the sole behavioral side-effects of STN stimulation despite good clinical improvement on long-term follow-up in patients and literature review suggests the complex role of STn stimulation in motor and behavioral control.
Abstract: Subthalamic nucleus (STN) stimulation is an established surgical treatment for Parkinson's disease (PD). Though the motor benefits of STN stimulation are well understood, its cognitive and behavioral effects are still not fully understood. Manic psychosis, hypersexuality, pathological gambling and mood swings are associated with advanced PD. There have been reports to suggest improvement or worsening in these symptoms following STN deep brain stimulation (DBS). We report two cases as the sole behavioral side-effects of STN stimulation despite good clinical improvement on long-term follow-up. These patients and literature review suggests the complex role of STN stimulation in motor and behavioral control.
45 citations
TL;DR: Dysarthria in PD, PSP and MSA have many overlapping but certain distinctive features as well which could serve as a diagnostic clue, and profound speech impairment probably indicative of the more severe frontostriatial pathology.
Abstract: Context: Speech abnormalities are common to the three Parkinsonian syndromes, namely Parkinson's disease (PD), progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), the nature and severity of which is of clinical interest and diagnostic value. Aim: To evaluate the clinical pattern of speech impairment in patients with PD, PSP and MSA and to identify significant differences on quantitative speech parameters when compared to controls. Design and Setting: Cross-sectional study conducted in a tertiary medical teaching institute. Materials and Methods: Twenty-two patients with PD, 18 patients with PSP and 20 patients with MSA and 10 age-matched healthy controls were recruited over a period of 1.5 years. The patients were clinically evaluated for the presence and characteristics of dysarthria. This was followed by quantitative assessment of three parameters: maximum phonation time (MPT), semantic fluency and reading speed. The outcome measures were compared between the patient groups and with controls. Results: Patients with PD had hypophonic monotonous speech with occasional rushes of speech while patients with MSA and PSP had mixed dysarthria with ataxic and spastic elements respectively. All quantitative parameters were affected when compared to controls ( P values <0.001, 0.012 and 0.008 respectively). Maximum phonation time was significantly less in PSP when compared to MSA and PD ( P =0.015). Reading speed also showed a similar trend which was not statistically significant. Semantic fluency was comparable in all three groups. Conclusion: Dysarthria in PD, PSP and MSA have many overlapping but certain distinctive features as well which could serve as a diagnostic clue. Patients with PSP had profound speech impairment probably indicative of the more severe frontostriatial pathology.
43 citations
TL;DR: Both techniques for hemispherotomy performed in patients with intractable epilepsies were equally effective, the procedure itself is very effective when indicated.
Abstract: Context: Hemispherotomy is a surgical procedure for hemispheric disconnection. It is a technically demanding surgery. Our experience is presented here. Aims: To validate and compare the two techniques for hemispherotomy performed in patients with intractable epilepsies. Settings and Design: A retrospective study 2001-March 2007: Nineteen cases of hemispherotomies from a total of 462 cases operated for intractable epilepsy. Materials and Methods: All the cases operated for intractable epilepsy underwent a complete epilepsy surgery workup. Age range 4-23 years (mean 5.2 years), 14 males. The seizure frequency ranged from 2-200 episodes per day; four were in status; three in epilepsia partialis continua. The pathologies included Rasmussen's, hemimegelencephaly (unilateral hemispheric enlargement with severe cortical and subcortical changes), hemispheric cortical dysplasia, post-stroke, post-traumatic encephalomalacia and encephalopathy of unknown etiology. The techniques of surgery included vertical parasaggital approach and peri-insular hemispherotomy. Neuronavigation was used in seven cases. Results: Class I outcome [Engel's] was seen in 18 cases and Class II in one assessed at 32-198 weeks of follow-up. The four patients in status epilepticus had Class I outcome. Four patients had an initial worsening of weakness which improved to preoperative level in five to eight weeks. Power actually improved in three other patients at 32-36 weeks of follow-up, but hand grip weakness persisted. In all the other patients, power continued to be as in preoperative state. Cognitive profile improved in all patients and 11 cases returned back to school. Conclusions: Both techniques were equally effective, the procedure itself is very effective when indicated. Four of our cases were quite sick and were undertaken for this procedure on a semi-emergency basis.
37 citations
TL;DR: Understanding the compensation of intracranial blood circulation, preoperative "Matas test" if it is necessary, perioperative hemodynamics testing and the application of revascularization techniques, will help reduce surgical risk and achieve a good surgical outcome.
Abstract: Aims: To investigate the characteristics and surgical treatment of traumatic intracranial pseudoaneurysms. Materials and Methods: Twelve patients with traumatic intracranial pseudoaneurysms were operated on in our hospital between 2000 and 2006. Their clinical characteristics, radiological features and surgical techniques were analyzed retrospectively. Four traumatic cavernous segment pseudoaneurysms underwent trapping of the internal carotid artery and others underwent neck reinforcement and clipping or crevasse clipping. Results: Nine patients were excellent or good and two patients were poor when they discharged. One patient died of postoperative cerebral infarction. Nine patients underwent follow-up (three months to seven years) and rebleeding was not seen in them. Conclusions: The surgical treatment of traumatic intracranial pseudoaneurysms is risky and difficult and individualized surgical option is necessary. Understanding the compensation of intracranial blood circulation, preoperative Matas test if it is necessary, perioperative hemodynamics testing and the application of revascularization techniques, will help reduce surgical risk and achieve a good surgical outcome.
36 citations
TL;DR: The authors report a case of pituitary macroadenoma in a 34-year-old female who underwent transsphenoidal tumor decompression and developed angiographically documented vasospasm during the course of her illness.
Abstract: Vasospasm, so commonly described after aneurysmal rupture, is very rare after surgery for brain tumors. Its occurrence after transsphenoidal surgery is extremely uncommon with only three cases reported as per the authors' review of the literature. The authors report a case of pituitary macroadenoma in a 34-year-old female who underwent transsphenoidal tumor decompression and developed angiographically documented vasospasm during the course of her illness. The rarity of the occurrence of vasospasm after transsphenoidal surgery makes its management very challenging, frequently leading to a delay in diagnosis and a poor outcome. The key to successful management is a high index of suspicion and early proactive management.
TL;DR: Alternative approaches of optimal utilization of rural, community health, satellite clinic and district models of the health care with close interaction with tertiary centres, nongovernmental agencies and private sector may facilitate achieving the goal of taking neurology care to the 'unreached'.
Abstract: Recognition of the magnitude of the burden and disability and mortality consequent to neurological disorders has led to global initiatives of declaring them as a "global epidemic", emphasizing public health approach and integration of neurology care with general health care. Epidemiological transition with increase in neurological disorders in India, the gross mismatch between the need and the available trained manpower and infrastructural facilities are posing challenges to health planners and policy makers for providing neurological care to the community. Alternative approaches of optimal utilization of rural, community health, satellite clinic and district models of the health care with close interaction with tertiary centres, nongovernmental agencies and private sector may facilitate achieving the goal of taking neurology care to the 'unreached'.
TL;DR: Percutaneous direct puncture embolization of cirsoid aneurysms is a safe and effective procedure and can be effectively used as an alternative to surgery.
Abstract: Background: Scalp is the most common site of soft tissue arteriovenous fistulae and surgical excision has been the primary mode of treatment. Endovascular treatment has evolved as an alternative to the surgery. Aims: To evaluate the effectiveness of percutaneous direct-puncture embolization of cirsoid aneurysms. Materials and Methods: From January 1995 to December 2004, 15 patients underwent percutaneous direct-puncture embolization of cirsoid aneurysms. Plain X-ray, computerized tomography scan and complete selective cerebral angiogram were done in all. Seven patients had forehead lesions, four had temporal and the remaining four patients had occipital region cirsoid aneurysms. Lesions were punctured with 21-gauge needle and embolized with 20-50% cyanoacrylate-lipiodol mixture. Circumferential compression was applied during injection. Results: Post-embolization angiogram showed complete obliteration in 11 patients. The remaining four patients required adjunctive transarterial embolization with polyvinyl alcohol particles for complete lesion devascularization. Two patients had post procedure surgery for removal of disfiguring and hard glue cast. There were no major procedure-related complications. No patients had any recurrence in the follow-up. Conclusion: Percutaneous direct puncture embolization of cirsoid aneurysms is a safe and effective procedure. It can be effectively used as an alternative to surgery. Sometimes adjunctive transarterial embolization is also required to deal with deeper feeders.
TL;DR: There was a single peak of age at onset at onset for males in the sixth and seventh decade and in females, in the third decade and the higher prevalence of thymomas in this series is in all probability related to selection bias as patients with thymic enlargement or more severe disease underwent thymectomy.
Abstract: Background: We present the findings from the largest hospital-based studies on myasthenia gravis from India, using data collected over a period of 43 years from the Neurology Department in a tertiary referral center in India. Objectives: To study the clinical presentation, age at onset, gender distribution, serological status and thymic pathology in patients with myasthenia gravis. Materials and Methods: A retrospective study was carried out using records of patients with myasthenia gravis from the years 1965 to 2008. Results: Of 841 patients, 836 (611 males and 225 females) had acquired myasthenia (myasthenia gravis) and five congenital myasthenia. The median age at onset was 48 years (males 53 years and females 34 years). The peak age at onset for males was in the sixth and seventh decade and in females, in the third decade. Two hundred and twenty-two (26.31%) patients had ocular and 616 (73.68%) generalized myasthenia. Serological studies were done in 281 patients with myasthenia gravis for Acetylcholine receptor (AchR) antibodies of which 238 (84.70%) were seropositive. The most common histopathology was thymoma and the second most common was thymic hyperplasia. Conclusion: Myasthenia gravis in our study was more common in males (M:F of 2.70:1). There was a single peak of age at onset (males sixth to seventh decade; females third decade). The higher prevalence of thymomas in this series is in all probability related to selection bias as patients with thymic enlargement or more severe disease underwent thymectomy. Thymoma was more common in males; hyperplasia in females.
TL;DR: Acute and/or subacute presentation of cryptococcal meningitis is not uncommon in HIV-infected individuals and an early diagnosis of HIV infection might reduce the incidence of this infection.
Abstract: Background: Cryptococcal meningitis is a common opportunistic infection in Human Immunodeficiency Virus (HIV)-infected individuals. There is little information specifically addressing cryptococcal meningitis in HIV-infected patients from North India. Aims: To determine clinical presentation, hospital course, response to treatment, complications developed, in-hospital mortality, any recurrence of cryptococcal meningitis and reasons of recurrence during follow-up. Settings and Design: A retrospective observational study undertaken in a large tertiary care center. Materials and Methods: Patient's demographic data, presenting clinical symptomatology, physical findings, laboratory parameters, cerebrospinal fluid (CSF) examination findings, side-effects of treatment, development of any complications and hospital outcome were analyzed. During follow-up any recurrence of cryptococcal meningitis, possible reasons of recurrence, type of treatment received, complications developed and outcome was recorded as well. Results: Forty patients diagnosed to have cryptococcal meningitis were analyzed. Twenty-two (55%) patients had acute/ subacute presentation. Thirty-six (90%) patients presented with headache and 18 (45%) had altered sensorium. Twenty (50%) patients had no cells in the CSF. Hypoglycorrhchia was seen in 30 (75%) patients. Cryptococcal meningitis was the first acquired immune deficiency syndrome (AIDS)-defining illness in 30 (75%) patients. Thirty-five patients developed some adverse effects to amphotericin-B. Thirty-three patients improved with treatment while three patients died. Four patients had recurrence of cryptococcal meningitis within six months of first episode. Non-compliance of fluconazole therapy was the reason for recurrence in all of these patients. Conclusions: Cryptococcal meningitis is a common initial AIDS-defining illness. Acute and/or subacute presentation of cryptococcal meningitis is not uncommon in HIV-infected individuals. An early diagnosis of HIV infection might reduce the incidence of this infection.
TL;DR: This study demonstrated that both the SF-36 and WHOQOL-100 quality of life scales are useful in the practical evaluation of patients with stroke.
Abstract: Background and Aims: Two widely used evaluation tools for the quality of life are the 36-item Short-Form Health Survey (SF-36) and World Health Organization Quality of Life Assessment (100-item version) (WHOQOL-100), however, these tools have not been compared for patients with stroke to date. The specific objectives of this study were: 1) to study the effect of stroke on quality of life (QOL) as measured by the SF-36 and by the WHOQOL-100, and 2) to compare these two instruments. Settings and Design: Seventy patients who were admitted to the neurology clinic six months after stroke were included in this study. Materials and Methods: As a data-collecting device, the SF-36 and WHOQOL-100 scales were used. An additional questionnaire was administered to obtain demographic data. Statistical Analysis: Pearson correlation analysis was performed and Blant-Altman Plots were used. Psychometric analysis was performed. Results: In stroke, the most flustered domains of quality of life were vitality and general health perception fields in the SF-36 and in the WHOQL-100, independence level field, overall QOL and general health perceptions. While there was a fair degree of relationship (r= 0.25-0.50) between general health perceptions, physical, social and mental fields that were similar fields of scales, a fair and moderate to good relationship was found between different fields. Limits of agreement in similar domains of the two instruments were very large. In all four demonstrated Bland-Altman plots, there was agreement of the scales in the measurements of similar fields of quality of life. Conclusion: This study demonstrated that both the SF-36 and WHOQOL-100 quality of life scales are useful in the practical evaluation of patients with stroke.
TL;DR: Cortical dysplasias have a good outcome if evaluated and managed with concordant electrical and imaging modalities.
Abstract: Background: There has been sparse description of cortical dysplasias (CDs) causing intractable epilepsy from India. Aim: Clinical retrospective study of CDs causing intractable epilepsy that underwent surgery. Materials and Methods: Fifty-seven cases of CDs reviewed (1995 till July 2006) are presented. All patients had intractable epilepsy, and underwent a complete epilepsy surgery workup (inter ictal electroencephalography (EEG), video EEG, MRI as per epilepsy protocol, SPECT {interictal, ictal with subtraction and co-registration when required}, and PET when necessary). Surgical treatment included a wide exposure of the pathology with a detailed electrocorticography under optimal anesthetic conditions. Mapping of the sensori-motor area was performed where indicated. Procedures included resection either alone or combined with multiple subpial transactions when extending into the eloquent areas. Results: Our study had 28 (49.12%) cases of isolated focal CDs, and 29 (50.67%) with dual pathology. Average age at the time of onset of seizures in our series was 7.04 years (three months to 24 years), and average age at the time of surgery was 10.97 years (eight months to 45 years). Among coexistent pathologies, one had associated MTS, 16 had coexistent gangliogliomas and 12 (dysembryonic neuroepithelial tumor) DNTs. At an average follow-up of 3.035 years (range 5-10 years), three patients were lost to follow-up. Fifty-one per cent (29/57) patients had a good outcome (Engel Grade I) and 26%(15/57) had a Grade II outcome. Conclusion: Cortical dysplasias have a good outcome if evaluated and managed with concordant electrical and imaging modalities.
TL;DR: A clinical trial has recently confirmed proof-of-principle that exclusion of Exon 51 from human dystrophin mRNAs, carrying frame-shifting deletions adjacent to this exon, results in dystrophic expression, and additional trials are underway, targeting the same exon but using an oligomer of different backbone chemistry.
Abstract: Duchenne muscular dystrophy (DMD), the most common and serious form of childhood muscle wasting is generally caused by protein-truncating mutations in the large DMD gene. Specific removal of an exon from a defective DMD gene transcript has the potential to allow synthesis of a semi-functional dystrophin, thereby reducing the severity and presumably progression of muscle wasting. The efficacy of this treatment will vary greatly between the different mutations that preclude the synthesis of a functional dystrophin. Restoration of the reading frame from a large multi-exon genomic deletion, typically greater than 36 exons, may lead to synthesis of a protein with only partial function and limited clinical benefit, whereas excising a nonsense mutation in a redundant exon should generate a near normal dystrophin. A clinical trial has recently confirmed proof-of-principle that exclusion of Exon 51 from human dystrophin mRNAs, carrying frame-shifting deletions adjacent to this exon, results in dystrophin expression. No major side-effects after local administration of the antisense oligomer were reported. Additional trials are underway, targeting the same exon but using an oligomer of different backbone chemistry. If functional dystrophin synthesis is demonstrated, and safety issues are addressed, subsequent trials will involve systemic delivery. Great challenges are ahead, some technical; establishing an effective delivery regimen, some ethical; choosing subsequent targets for therapy, and others of an administrative and regulatory nature.
TL;DR: Though the exact mechanism of LRPN in diabetic and non-diabetic patients remains unknown, new evidence alludes to an underlying inflammatory vasculitic process.
Abstract: Background: Lumbosacral radiculoplexus neuropathy (LRPN) originally described in diabetic patients is a distinct clinical condition characterized by debilitating pain, weakness and atrophy most commonly affecting the proximal thigh muscles asymmetrically. The syndrome is usually monophasic and preceded by significant weight loss (at least more than 10 lbs). Though a self-limited condition, recovery is gradual with some residual weakness. Recent advances and research has provided new insights in the pathogenesis and thereby management of this syndrome. In this paper, we review the clinical and diagnostic features as well as discuss recent insights and treatment strategies along with our experience in the management of patients with diabetic and non-diabetic LRPN. Materials and Methods: Literature in English published between 1953 and 2008 was searched in the MEDLINE and Pubmed database, maintained by the US National library of medicine and National institutes of health, using key words of diabetic amyotrophy, lumbosacral radiculoplexus neuropathy, diabetic proximal neuropathy, diabetic radiculopathy and diabetic lumbosacral plexopathy. In addition, literature reported in various textbooks on peripheral neuropathy was reviewed as well. Observation: The diagnosis relies mostly on clinical suspicion and characteristic electromyographic findings. The exact pathogenesis of the illness remains unknown, but there seems to be a component of immune-mediated inflammatory microvasculitis which causes secondary ischemia of the lumbosacral plexus. This has prompted a trial of immunosuppressive agents (like steroids) with an attempt to alter the course of the illness. A few reports have noted that immunosuppression when instituted early in the course of the illness (within three months of symptom onset) may hasten the recovery and improve symptoms. Conclusion: Though the exact mechanism of LRPN in diabetic and non-diabetic patients remains unknown, new evidence alludes to an underlying inflammatory vasculitic process. Early treatment with immunosuppressants may be beneficial in some cases, although the data available at this time is limited to a small cohort of patients. The decision is individualized weighing the risks and benefits in a given patient. Future research in this direction with double-blinded case-controlled studies is required to investigate this further.
TL;DR: The study aimed at correlation of post-stroke dysphagia with area and volume of infarct/ bleed, and with subsequent in-hospital respiratory morbidity and mortality.
Abstract: Aims: The study aimed at correlation of post-stroke dysphagia with area and volume of infarct/ bleed, and with subsequent in-hospital respiratory morbidity and mortality. Materials and Methods: 50 patients of acute stroke were serially recruited. Standard Staff swallowing assessment was performed within 24 hours of admission along with pulse oximetry. Ischemic strokes were classified as per OCPS registry. In-hospital respiratory morbidity and mortality, mode of nutrition and disability status at discharge were noted. Results: 21/50 (42%) patients had post-stroke dysphagia during their hospital course. Among infarcts, Total Anterior Circulation Infarcts (TACI) had 100% incidence of dysphagia, followed by Partial Anterior Circulation Infarcts (PACI-36%), Posterior Circulation infarcts (POCI-33%), and Lacunar infarcts (LACI-18%). 67% of hemorrhages had post-stroke dysphagia. Staff swallowing assessment had a sensitivity and specificity of 75% and 73% respy., for predicting respiratory morbidity. The corresponding figures for Pulse oximetry were 79% and 91%.
TL;DR: Endoscopic aspiration of brain abscess appears to be a safe and effective alternative method of treatment, there is direct visualization of abscess cavity, completeness of aspiration can be assessed, and perioperative bleeding can be controlled.
Abstract: Background: Treatment of brain abscess is still a subject of controversy. Simple therapeutic approaches like twist drill/burr hole aspiration with or without insertion of a drain are also quite effective. There are reports of encouraging results following endoscopic treatment. We are reporting our results of endoscopic approach on 24 patients. Materials and Methods: This is a prospective study on 24 patients of brain abscesses treated between January 2004 and January 2007. All the cases except those with small abscesses (less than 1.0 cm in diameter) and multiloculated abscesses were included. Gabb 6-degree rigid endoscope was used. Repeat CT scan was done in all cases within 7 and 30 days after surgery. Ten patients (42%) had small residual abscess on 7 th post-operative day's CT scan, while 30 th post-operative day's CT scan did not show any significant lesion in all the cases. Results: There were 23 patients of chronic otitis media and one of congenital cyanotic heart disease. Glasgow coma score (GCS) was 3 in one patient, 13 in two cases, 14-15 in 21 cases. There were 14 cerebellar, 8 temporal and 1 frontal and thalamic abscess each. All the patients recovered completely except one who died (GCS 3). There was no procedure-related complication. Hospital stay ranged from 7 to 12 days with an average of 8.2 days. Follow-up ranged between 6 and 42 months. Conclusion: Endoscopic aspiration of brain abscess appears to be a safe and effective alternative method of treatment. There is direct visualization of abscess cavity, completeness of aspiration can be assessed, and perioperative bleeding can be controlled.
TL;DR: The successful treatment of a patient with aspergillus sellar abscess, a 65-year-old woman presented with headache, nasal discharge and decreased visual acuity, is described.
Abstract: Aspergillus sellar abscess is a very rare form of fungal infections of the central nervous system (CNS). In this report, we describe the successful treatment of a patient with aspergillus sellar abscess. A 65-year-old woman presented with headache, nasal discharge and decreased visual acuity. The diagnosis of sellar mass was made on the basis of magnetic resonance imaging (MRI) examination. The computed tomography (CT) scan revealed sellar enlargement and sellar floor bony destruction. After hospitalization the patient underwent transsphenoidal surgery. Histopathological examination of the sellar mass revealed aspergillosis. Postoperatively, amphotericine-B and itraconazole therapy was started. During a six-month follow-up, the patient's headache and inertia disappeared, visual acuity improved. Aspergillus sellar abscess must be considered in the differential diagnosis of a sellar mass. The correct diagnosis of pituitary aspergillosis can only be achieved by histopathological examination. Surgical intervention and antifungal therapy should be considered the optimal treatment.
TL;DR: The clinical features in 28 patients with dysferlin deficiency confirmed by muscle immunohistochemistry (IHC) were onset in late adolescence or early adulthood and Dysferlinopathies was not an uncommon form of LGMD.
Abstract: Background: Miyoshi myopathy (MM) and limb girdle muscular dystrophy (LGMD2B) are distinct clinical entities because different muscle groups are involved at the onset. We describe the clinical features in 28 patients with dysferlin deficiency confirmed by muscle immunohistochemistry (IHC). Settings and Design: A case series from a tertiary national referral center for neurological disorders. Materials and Methods: Patients with classical phenotype of MM and LGMD2B underwent a thorough phenotypic characterization followed by muscle histopathological study including IHC for dysferlin deficiency. Results: There were 28 patients (20 men and eight women) presenting with manifestations of distal myopathy or LGMD2B and had absence of dysferlin staining on IHC. Patients presented predominantly with distal myopathy of Miyoshi type (MM) or proximal LGMD type and were diagnosed to have dysferlinopathy on IHC. Two patients had the proximodistal form and two had onset as tibial muscular dystrophy. The main clinical features in these patients were onset in late adolescence or early adulthood (mean age of onset for MM was 22.0 ± 6.7 years and for LGMD2B 19.4 ± 5.1 years). There was early and predominant involvement of the posterior compartment muscles of the leg or proximal pelvic girdle muscles, dystrophic features with necrotic regeneration pattern without vacuoles on muscle biopsy and markedly elevated serum creatine kinase values with mean of 10033.8 ± 9283 IU/l (range 402-27460). Consanguinity was reported in 46.4%. The mean duration of illness was 6.4 ± 4.2 years. Dysferlinopathies formed nearly one-fourth of our patients with LGMD. Conclusion: In our experience dysferlinopathies was not an uncommon form of LGMD.
TL;DR: The presented fiber dissection study clarifies the relationship of optic radiation and five previously frozen and formalin-fixed cadaveric human brains were used.
Abstract: Objective: Using a fiber-dissection technique, our aim is to study the fiber bundles of the optic radiation. We focused on the course, the length, anatomical relations with lateral ventricle and the relevance of these finding during surgery in the region. Materials and Methods: Five previously frozen and formalin-fixed cadaveric human brains were used. The dissection was done using the operating microscope. Fiber dissection techniques described by Klingler were adopted. The primary dissection tools were handmade, thin, and wooden and curved metallic spatulas with tips of various sizes. Lateral and inferior temporal approaches were made and the optic fiber tracts were dissected. Results: Resections that extend through the roof of the temporal horn more than 30 mm behind the temporal pole cross the Meyer's loop. In the middle temporal gyrus approach, resection that is taken through the lateral wall of the temporal horn more than 55-60 mm behind the temporal pole may cross the optic radiation during their course here on the lateral wall. Conclusion: The presented fiber dissection study clarifies the relationship of optic radiation. Such fiber dissection studies are only few in the literature.
TL;DR: The diagnostic process by which limb girdle muscular dystrophy disorders can be defined and the implications of making these diagnoses are concentrated on.
Abstract: The increasing knowledge about limb girdle muscular dystrophy (LGMD) has clarified many aspects of this extensive group of neuromuscular conditions and has moreover proven their wide clinical and genetic heterogeneity. For these reasons, achieving a precise diagnosis of a particular type of LGMD may be still difficult and requires a comprehensive approach, which includes epidemiology, medical history, clinical examination, laboratory and genetic tests. All of the LGMDs are individually rare and their population frequency is highly variable. The prevalence of the different forms of LGMD in different populations has to be considered for the differential diagnosis. Some characteristic clinical features may help to distinguish subtypes of LGMD however protein analysis on muscle biopsy and genetic testing still represent the gold standard in the diagnosis of these muscular dystrophies. Reaching a precise diagnosis in all patients is important to allow genetic counseling to be properly applied and to direct appropriate medical management with a potential positive impact on the length and quality of life. Moreover, new specific therapeutic approaches, including limited local gene therapy, have been emerging over the last few years and require a precise genetic definition of the conditions. This article will concentrate on the diagnostic process by which these disorders can be defined and the implications of making these diagnoses.
TL;DR: Asleep-awake-asleep technique using laryngeal mask airway offers airway protection during awake craniotomy with or without securing airway, and patients remained awake and cooperative throughout the time of neurological testing.
Abstract: Asleep-awake-asleep technique of anesthesia is used during awake craniotomy with or without securing airway. We assessed this technique using laryngeal mask airway (LMA) in two patients. Patients underwent awake craniotomy for epilepsy surgery and the removal of a frontotemporal glioma. After anesthesia induction, airway was secured using LMA. Anesthesia was maintained using oxygen, nitrous oxide and sevoflurane, supplemented with an infusion of propofol and remifentanil. Twenty minutes before corticography, anesthesia was discontinued and LMA removed. Both patients were awake and cooperative during the neurological assessment and surgery on eloquent areas. The LMA was reinserted before the closure of the dura and remained in place until the end of surgery. Both patients had no recall of events under anesthesia, although experienced mild pain and discomfort during awake phase of surgery. Both expressed complete satisfaction over the anesthetic management. Asleep-awake-asleep technique using LMA offers airway protection. The painful aspect of surgery can be performed under anesthesia, hence minimizing the duration of stress and pain. Patients remained awake and cooperative throughout the time of neurological testing.
TL;DR: This significant gene deletion analysis has been carried out for Becker muscular dystrophy patients particularly from Western India using 32 exons.
Abstract: Background: Becker muscular dystrophy (BMD) is caused by mutations in the dystrophin gene with variable phenotypes. Becker muscular dystrophy patients have low levels of nearly full-length dystrophin and carry in-frame mutations, which allow partial functioning of the protein. Aim: To study the deletion patterns of BMD and to correlate the same with reading frame rule and different phenotypes. Setting: A tertiary care teaching hospital. Design: This is a prospective hospital-based study. Materials and Methods: Thirty-two exons spanning different hot spot regions using Multiplex PCR techniques were studied in 347 patients. Two hundred and twenty-two showed deletions in one or more of the 32 exons. Out of these, 46 diagnosed as BMD patients were analyzed. Results: Forty-six BMD patients showed deletions in both regions of the dystrophin gene. Out of these 89.1% (41/46) were in-frame deletions. Deletions starting with Exon 45 were found in 76.1% (35/46) of the cases. Mutations in the majority of cases i.e. 39/46 (84.8%) were seen in 3' downstream region (Exon 45-55, distal rod domain). Few, i.e. 5/46 (10.8%) showed deletions in 5' upstream region (Exons 3-20, N-terminus and proximal rod domain) of the gene, while in 2/46 (4.4%) large mutations (>40 bp) spanning both regions (Exons 3-55) were detected. Conclusion: This significant gene deletion analysis has been carried out for BMD patients particularly from Western India using 32 exons.
TL;DR: It is more likely to relieve pain in patients with recurrent or refractory TN who did not have a prior procedure or who had fewer than three procedures before undergoing their first GKRS.
Abstract: Background: Repeat gamma knife radiosurgery (GKRS) is considered to be an effective treatment for refractory or recurrent trigeminal neuralgia (TN). Aims: The purpose of this report was to demonstrate the relationship between the outcome of repeat GKRS and prior operative procedures on patients with recurrent or refractory TN. Materials and Methods: A retrospective analysis was performed on 34 patients with refractory or recurrent TN who had undergone repeat GKRS; 21 patients had undergone other types of procedures, 11 of whom had undergone more than three such procedures prior to radiosurgery. The maximum dose of the repeat procedure was between 60 and 75 Gy. The mean follow-up time was 21.6 months. Statistical Analysis Used: The log-rank test and Fisher's exact test were used to analyze the data. Results: Excellent pain relief was achieved in 14 patients (41.2%) after repeat GKRS, while a successful outcome occurred in 29 of 34 patients (85.3%). Better pain relief occurred in the patients who did not have a prior procedure or who had undergone fewer than three prior procedures ( P = 0.042). Twenty-four of 25 patients (96.0%) who had recurrent pain had a successful operation and five of nine patients (55.6%) who did not have significant relief of pain after the first procedure had a successful operation. The difference was statistically significant ( P < 0.01). Only four patients had mild complications. Conclusion: It is more likely to relieve pain in patients with recurrent or refractory TN who did not have a prior procedure or who had fewer than three procedures before undergoing their first GKRS. Moreover, it seems that patients who had a good response following the initial GKRS had better results after a repeat procedure.
TL;DR: MHC Class I immunostaining can be used as a complementary diagnostic tool for the diagnosis of idiopathic inflammatory myopathies.
Abstract: Aim: Utility of major histocompatibility complex (MHC) Class I antigen immunostaining was studied to differentiate idiopathic inflammatory myopathies from dystrophies. Materials and Methods: Forty muscle biopsies including seven dermatomyositis (DM), six polymyositis (PM), two sporadic inclusion body myositis (sIBM), 20 dystrophies (one Duchenne, three Becker's, four alpha, one gamma sarcoglycanopathy, nine limb girdle, one myotonic and one fascioscapulohumeral muscular dystrophy) and five controls were stained with antibody for MHC Class I antigen (Novocastra clone W6/32 HL 1:100 dilution). Results: Polymyositis and sIBM showed MHC class I antigen positivity along sarcolemma of single and small groups of muscle fibers. The regenerating fibers in the perifascicular area in DM showed intense cytoplasmic positivity of MHC class I antigen. Muscle fibers in all dystrophies except regenerating fibers and control normal muscle were negative for MHC. Capillaries and lymphocytes were positive controls. There were no false positives in the study. Conclusion: MHC Class I immunostaining can be used as a complementary diagnostic tool for the diagnosis of idiopathic inflammatory myopathies.
TL;DR: The neuromuscular junction (NMJ) is a specialized synapse with a complex structural and functional organization that is a target for a variety of immunological disorders and these diseases usually respond well to immunotherapies.
Abstract: The neuromuscular junction (NMJ) is a specialized synapse with a complex structural and functional organization. It is a target for a variety of immunological disorders and these diseases usually respond well to immunotherapies. The understanding of the immunological basis of myasthenia gravis, the most common neuromuscular junction disorder, has improved in the recent years. Most patients have antibodies to the acetylcholine receptor (AChR), but around 10% have AChR antibodies that are only identified by novel methods, and up to 5% have muscle-specific kinase antibodies which define a different subgroup of myasthenia. The spectrum of antibodies and their pathophysiological aspects are being elucidated. Even though less common, Lambert Eaton myasthenic syndrome (LEMS) is important to recognize. The abnormality in LEMS is a presynaptic failure to release enough packets of ACh, caused by antibodies to the presynaptic voltage-gated calcium channels. More than half these patients have a small cell carcinoma of lung. Acquired neuromyotonia (NMT) is a condition associated with muscle hyperactivity. Clinical features include muscle stiffness, cramps, myokymia, pseudomyotonia and weakness. The immune mechanisms of acquired NMT relate to loss of voltage-gated potassium channel function. This review will focus on the important recent developments in the immune-mediated disorders of the NMJ.