Showing papers in "Neurology India in 2009"

Neurological complications of Chikungunya virus infection.

Abstract: Background: In May 2006, there was a large Chikungunya virus infection (CHIKV) outbreak in the Nagpur district of Maharashtra, a province in western India. Usually, CHIKV is a self-limiting febrile illness. However, neurological complications have been described infrequently. Aim: To study the clinical characteristics of various neurological complications associated with CHIKV infections. Materials and Methods: Patients with neurological complications following CHIKV infection during the outbreak were the subjects of the study. On the basis of clinical features and investigative findings, patients were grouped into various neurological syndromes: Encephalitis, myelopathy, peripheral neuropathy, myeloneuropathy, and myopathy. Cerebrospinal fluid (CSF) samples were also collected for biochemical and serological studies. Results: Of the 300 patients with CHIKV infection seen during the study period, June-December 2006, 49 (16.3%) [M : F: 42:7] had neurological complications. The neurological complications included: Encephalitis (27, 55%), myelopathy (7, 14% ), peripheral neuropathy (7, 14%), myeloneuropathy (7, 14%), and myopathy (1, 2%). Reverse Transcriptase polymerase chain reaction (RT-PCR) and real-time PCR was positive in the CSF in 16% and 18%, respectively. Conclusion: Recent CHIKV infection was associated with various neurological complications, suggesting neurotropic nature of the virus. The outcome of the neurological complications is likely to be good.

Etiological factors of traumatic peripheral nerve injuries.

Abstract: Background: Traumatic injury of peripheral nerves is a worldwide problem and can result in significant disability. Management of peripheral nerve injuries (PNIs) requires accurate localization and the assessment of severity of the lesion. Aim: The purpose of this study is to analyze the data of patients with PNIs referred for electromyography to a tertiary care hospital. Materials and Methods: This is a retrospective study of clinical and electromyographic data of patients with PNIs seen over a period of eight-years (1999-2007) in a tertiary hospital. The data collected included: Demographic data, cause, type of lesion, anatomical location of the lesion, and the mechanism of lesion. Results: During the study period 938 patients were seen with nerve injuries and the distribution of nerve injuries was: PNIs: 1,165; brachial plexus lesions: 76; and lumbar plexus lesions: 7. The mean age was 31.8 years (range 2-81 years) and the male to female ratio was 2.4:1. The most frequent nerve injuries were ulnar nerve in the upper extremity and sciatic nerve in the lower extremity. The most common cause of nerve injury was motor vehicle accidents. Two-thirds of the PNIs were partial. Conclusion: This study can serve as a guide to determine the epidemiology and classification of traumatic peripheral and plexus injuries.

Management of hydrocephalus in patients with tuberculous meningitis

Abstract: Hydrocephalus is one of the commonest complications of tuberculous meningitis (TBM) occurring in up to 85% of children with the disease. It is more severe in children than in adults. It could be either of the communicating type or the obstructive type with the former being more frequently seen. The Vellore grading system for clinical grading of patients with TBM and hydrocephalus with grade I being the best grade and grade IV being the worst grade has been validated by several authors. The management of hydrocephalus can include medical therapy with dehydrating agents and steroids for patients in good grades and those with communicating hydrocephalus. However, surgery is required for patients with obstructive hydrocephalus and those in poor grades. Surgery can involve either a ventriculo-peritoneal shunt or endoscopic third ventriculostomy (ETV). Complications of shunt surgery in patients with TBM and hydrocephalus are high with frequent shunt obstructions and shunt infections requiring repeated revisions. ETV has variable success in these patients and is generally not advisable in patients in the acute stages of the disease. Mortality on long-term follow up has been reported to vary from 10.5% to 57.1% in those with altered sensorium prior to surgery and 0 to 12.5% in patients with normal sensorium. Surgery for patients in Vellore grade IV is usually associated with a poor outcome and high mortality and therefore, its utility in these patients is debatable.

Basilar invagination, Chiari malformation, syringomyelia: a review.

Abstract: Institute and personal experience (over 25 years) of basilar invagination was reviewed. The database of the department included 3300 patients with craniovertebral junction pathology from the year 1951 till date. Patients with basilar invagination were categorized into two groups based on the presence (Group A) or absence (Group B) of clinical and radiological evidence of instability of the craniovertebral junction. Standard radiological parameters described by Chamberlain were used to assess the instability of the craniovertebral junction. The pathogenesis and clinical features in patients with Group A basilar invagination appeared to be related to mechanical instability, whereas it appeared to be secondary to embryonic dysgenesis in patients with Group B basilar invagination. Treatment by facetal distraction and direct lateral mass fixation can result in restoration of craniovertebral and cervical alignment in patients with Group A basilar invagination. Such a treatment can circumvent the need for transoral or posterior fossa decompression surgery. Foramen magnum bone decompression appears to be a rational surgical treatment for patients having Group B basilar invagination. The division of patients with basilar invagination on the basis of presence or absence of instability provides insight into the pathogenesis of the anomaly and a basis for rational surgical treatment.

Management of the trigeminocardiac reflex: facts and own experience.

Abstract: The trigeminocardiac reflex (TCR) is defined as the sudden onset of parasympathetic dysrhythmia, sympathetic hypotension, apnea, or gastric hyper-motility during stimulation of any of the sensory branches of the trigeminal nerve. The proposed mechanism for the development of TCR is--the sensory nerve endings of the trigeminal nerve send neuronal signals via the Gasserian ganglion to the sensory nucleus of the trigeminal nerve, forming the afferent pathway of the reflex arc. It has been demonstrated that the TCR may occur with mechanical stimulation of all the branches of the trigeminal nerve anywhere along its course (central or peripheral). The reaction subsides with cessation of the stimulus. But, some patients may develop severe bradycardia, asystole, and arterial hypotension which require intervention. The risk factors already known to increase the incidence of TCR include: Hypercapnia; hypoxemia; light general anesthesia; age (more pronounced in children); the nature of the provoking stimulus (stimulus strength and duration); and drugs: Potent narcotic agents (sufentanil and alfentanil); beta-blockers; and calcium channel blockers. Because of the lack of full understanding of the TCR physiology, the current treatment options for patients with TCR include: (i) risk factor identification and modification; (ii) prophylactic measures; and (iii) administration of vagolytic agents or sympathomimetics.

Neurology of endemic skeletal fluorosis.

Abstract: Endemic skeletal fluorosis is widely prevalent in India and is a major public health problem. The first ever report of endemic skeletal fluorosis and neurological manifestation was from Prakasam district in Andhra Pradesh in the year 1937. Epidemiological and experimental studies in the endemic areas suggest the role of temperate climate, hard physical labor, nutritional status, presence of abnormal concentrations of trace elements like strontium, uranium, silica in water supplies, high fluoride levels in foods and presence of kidney disease in the development of skeletal fluorosis. Neurological complications of endemic skeletal fluorosis, namely radiculopathy, myelopathy or both are mechanical in nature and till date the evidence for direct neurotoxicity of fluoride is lacking. Prevention of the disease should be the aim, knowing the pathogenesis of fluorosis. Surgery has a limited role in alleviating the neurological disability and should be tailored to the individual based on the imaging findings.

Assessment of microscope-integrated indocyanine green angiography during intracranial aneurysm surgery: A retrospective study of 120 patients

Abstract: Aims: The purpose of this study was to assess the clinical value of indocyanine green angiography (ICG) in intracranial aneurysm surgery by comparing the findings with postoperative angiographic results. Materials and Methods: One hundred and twenty patients with 148 intracranial aneurysms were included. ICG angiography was performed before and/or after the aneurysm clipping. A near-infrared excitation light illuminated the operation field, ICG was injected intravenously. The intravenous fluorescence was imaged with a video camera integrated into the microscope. Results: A total of 208 investigations of ICG angiography were performed. Aneurysm clipping was applied in 120 patients. Incomplete clipping was detected in four patients. Parent and/or branching artery stenosis was found in five patients. Delayed perfusion of ICG was detected in one patient. Postoperative digital subtraction angiography (DSA) was performed in 108 patients. The postoperative angiographic results were consistent with findings on intraoperative ICG angiograms in 100 patients (92.6%). In three cases, a mild stenosis was seen on DSA, which was not detected intraoperatively using ICG angiogram. In one patient, middle cerebral artery stenosis was found. Three patients had small residual aneurysms found by postoperative DSA. The remaining one developed a severe cerebral vasospasm. Conclusions: ICG angiography is a simple, reliable and cost-effective method. It provides real-time information in detecting the patency of parent, branching, perforating arteries and residual aneurysm. This technique may be a useful adjunct to improve the quality of intracranial aneurysm surgery.

Neurology of acute organophosphate poisoning.

Abstract: Acute organophosphate (OP) poisoning is one of the most common poisonings in emergency medicine and toxicological practice in some of the less-developed nations in South Asia. Traditionally, OP poisoning comes under the domain of emergency physicians, internists, intensivists, and toxicologists. However, some of the complications following OP poisoning are neurological and involve neurologists. The pathophysiological basis for the clinical manifestations of OP poisoning is inactivation of the enzyme, acetylcholinesterase at the peripheral nicotinic and muscarinic and central nervous system (CNS) nerve terminals and junctions. Nicotinic manifestations occur in severe cases and late in the course; these comprise of fasciculations and neuromuscular paralysis. There is a good correlation between the electrophysiological abnormalities and the severity of the clinical manifestations. Neurophysiological abnormalities characteristic of nicotinic junctions (mainly neuromuscular junction) dysfunction include: (1) single, supramaximal electrical-stimulus-induced repetitive response/s, (2) decrement-increment response to high frequency (30 Hz) repetitive nerve stimulation (RNS), and (3) decremental response to high frequency (30 Hz) RNS. Atropine ameliorates muscarinic manifestations. Therapeutic agents that can ameliorate nicotinic manifestations, mainly neuromuscular, are oximes. However, the evidence for this effect is inconclusive. This may be due to the fact that there are several factors that determine the therapeutic effect of oximes. These factors include: The OP compound responsible for poisoning, duration of poisoning, severity of poisoning, and route of exposure. There is also a need to study the effect of oximes on the neurophysiological abnormalities.

Wilson's disease: An Indian perspective.

Abstract: Wilson's disease (WD) is an autosomal recessive disease involving a defect of copper transport by the hepatic lysosomes. It leads to excess copper deposition in the liver, the brain, the kidneys and the skeletal system, affecting most commonly children or young adults and running an invariably fatal course if not adequately treated by de-coppering therapy. The last century has witnessed several changes, notable among these are: Increased awareness, improved diagnostic facilities leading to earlier recognition even in the pre-symptomatic phase, clear distinction from its mimics, aggressive therapeutic approaches owing to availability of effective treatment and an overall reduction in the morbidity and mortality. It is widely acknowledged that the disease is not as rare as once believed. Sir SAK Wilson published his landmark article in 1912, but it was only in 1968 that the first patient of WD was reported from our country. Publications from India on WD have focused on phenotypic characterization, documentations of lesser recognized aspects of the disease e.g. seizures, behavior abnormality, speech and cognitive impairment, sub-clinical affection of visual pathway, heart and autonomic function and pre-symptomatic detection. Attempts have been made to understand the clinical heterogeneity of the disease through identification of biochemical and immunological markers, magnetic resonance imaging, neuropathological study and genetic analysis for novel and/or known mutations. Assessment of impairment and severity and effect of various therapeutic interventions namely zinc sulphate on the long-term outcome and quality of life have also been studied. Nevertheless, clinicians often face difficulties in long-term care of these patients. Diagnostic errors leading to delay in diagnosis and initiation of treatment are common, even in patients with positive family history. There is no consensus regarding therapeutic protocols since the use of penicillamine, once a 'gold standard' for treatment, has been debated by experts. Mortality and morbidity of this potentially treatable disease and nonavailability of medications to the poor patients remain a major area of concern.

Microvascular decompression as a surgical management for trigeminal neuralgia: a critical review of the literature.

Abstract: Trigeminal neuralgia (TN) is a common pain syndrome and is characterized by recurrent episodes of intense lancinating pain in one or more divisions of the trigeminal nerve. Neurovascular compression (NVC) has been considered as the main cause of TN in the root entry zone (REZ) of the trigeminal nerve in the cerebellopontine angle cistern. Microvascular decompression (MVD) is the surgical procedure of choice for the treatment of medically refractory TN. MVD has also been shown to provide pain relief even in patients without visible neurovascular compression. Additionally, it has been accepted that MVD can provide the highest rate of long-term patient satisfaction with the lowest rate of pain recurrence. We did, systematic review of the subject and also our own experiences.

Transorbital penetrating cerebral injury with a ceramic stone: report of an interesting case.

Abstract: Penetrating cranial injury is a potentially life-threatening condition. The majority of war injuries are high-velocity penetrating cranial injuries; but in civilian cases, most penetrating cranial wounds are low-velocity type. We report an interesting case of transorbital penetrating cranial injury with a knife-sharpening stone made up of ceramic in a 28-year-old male. The pertinent literature is reviewed and management of such cases is discussed.

Acute ischemic infarct in the middle cerebral artery territory following a Russell's viper bite.

Abstract: Ischemic stroke following snake bite is rare. We report an 18-year male who developed right hemiplegia with expressive aphasia following a Russell's viper bite. T2-weighted magnetic resonance imaging revealed infarct in the left middle cerebral artery territory. The possible mechanisms for cerebral infarction in this scenario include disseminated intravascular coagulation, toxin induced vasculitis and endothelial damage.

"Free-hand" technique for thoracolumbar pedicle screw instrumentation: Critical appraisal of current "State-of-Art"

Abstract: Pedicle screws are widely used for instrumentation of the thoracolumbar spine. The anatomic studies performed in the last two decades, detailing the complex morphometry and three-dimensional anatomy of the thoracolumbar pedicles, have enabled the emergence of the so-called "free-hand" technique of pedicle screw placement based exclusively on anatomical parameters. However, in the thoracic spine, the benefits of pedicle screws have been tempered by its potential risks, such as, spinal canal violation, pedicle fracture, nerve root compression, and vascular lesions. Furthermore, the narrow and inconsistent shape of the thoracic pedicles, especially in spinal deformity, makes their placement technically challenging. In this article, the authors make a critical appraisal of current "state-of-art" of "free-hand" technique of pedicle instrumentation, analyzing its anatomical basis, surgical technique, present indications and limitations as well as the role of adjuvant image-guided and neurophysiological monitoring methods.

Challenges in neurosurgical intraoperative consultation.

Abstract: Background: Intraoperative consultation for neurosurgical specimens can be difficult at times, despite the use of both frozen section and squash preparation. Various factors influence the diagnostic accuracy of these procedures. This study was conducted to evaluate reasons for discordant case results in neurosurgical intraoperative consultations and make a comparative analysis of these two commonly used methods to identify the possible pitfalls, errors, and limitations. Materials and Methods: All the neurosurgical cases received in the Department of Pathology for intraoperative consultation over a period of 3 years were studied retrospectively. The slides of frozen sections and squash preparation were retrieved and the diagnosis was compared with the final diagnosis given on paraffin-embedded sections. Results and Observations: A total of 6% of the cases were found to be discordant; these included angiomatous meningioma, Non-Hodgkins lymphoma, metastatic renal cell carcinoma, cerebellopontine angle fibrous meningioma, and craniopharyngioma. Highly vascular lesions, unavailability of squash preparation in a few cases and technical errors like thick smears, excessively crushed specimen, freezing, and cautery induced and crushing artifacts contributed to misdiagnosis. Conclusion: The discrepant cases need to be reviewed regularly by pathologists to familiarize themselves with the morphological changes and artifacts. The knowledge of possible errors could minimize misinterpretation and help in providing a more conclusive opinion to the operating surgeon.

Calvarial tuberculosis: A report of eleven patients

Abstract: Background : Tuberculosis is endemic in developing countries. However, calvarial tuberculosis is rare, even in areas where tuberculosis is endemic. In the literature, only few case series of calvarial tuberculosis have been reported. Aim : To report a case series of 11 patients with calvarial tuberculosis, and discuss their presentation and management. Materials and Methods : This study is a retrospective analysis of case records of 11 patients with calvarial tuberculosis treated between 2001 and 2005 in a tertiary hospital. Clinical features, radiological findings, surgical and medical management, and outcomes were reviewed. Results : Of the 11 patients, seven were male and the age ranged between 1 and 25 years (mean 15.09 years). The mean duration of symptoms was 2.9 months (range 1-5 months). The most common presenting features were scalp swelling, discharging sinus, and pain. Computed tomography (CT) scan of brain showed punched out bony defect, with a peripherally enhancing extradural collection in most of the cases. Ten patients underwent surgical excision of necrotic bone and granulation tissue with primary closure of the scalp flap and antituberculous therapy. One patient is being managed with antituberculous therapy only. Of the 10 patients treated surgically and with antituberculous therapy, nine recovered well and one died of tuberculous meningitis and hydrocephalus. The patient being treated with antituberculous therapy is under follow-up. Conclusion : A high index of suspicion and knowledge is required for early diagnosis of calvarial tuberculosis. Surgery and antituberculous therapy remains the mainstay of treatment.

Evaluation of nine children with reversible posterior encephalopathy syndrome.

Abstract: Background: Reversible posterior leukoencephalopathy syndrome (PRES) is a neurological disorder characterized by signs of posterior cerebral edema upon radiographic examination. Materials and Methods: We retrospectively analyzed the records of nine children with the diagnosis of PRES. Results: Of the nine patients, seven were receiving immunosuppressive therapy and two were acute hypertensive crisis associated with renal disease. Immunosupressive drugs were intrathecal methotrexate in two patients, cyclosporine in two patients, intrathecal cytarabine in one patient, cyclophasphamide in one patient, and intravenous immunoglobulin (IVIg) in another one patient. The most presenting symptoms were seizure, headache, and altered consciousness. Six patients had seizures. Altered consciousness was present in four patients. Headache and nausea or vomiting was present also in six patients. Visual abnormalities were noted in two patients. Magnetic resonance imaging (MRI) studies showed white-matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres, but the changes often involved other cerebral areas, the brain stem, basal ganglia or the cerebellum. The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn. In all the patients, the clinical and radiological findings resolved morly completely. Conclusion: Reversible posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed. This syndrome should be recognized immediately and trigger agents can be discontinued to prevent long-term sequelae.

D-dimer is useful in the diagnosis of cortical venous sinus thrombosis.

Abstract: Background: Estimations of D-dimer correlate with deep vein thrombosis and pulmonary thromboembolism and may serve as a marker of cortical venous sinus thrombosis (CVST). Aim: To study the usefulness of D-dimer in the diagnosis of CVST. Materials and Methods: A total of 26 patients with CVST were subjected to a detailed medical history and neurological evaluation. A cranial MRI was carried out on a 1.5T scanner using T1, T2, and DWI sequences and two-dimensional time of flight MR venography. D-dimer was estimated using a rapid latex agglutination slide test using monoclonal antibodies. Results: The age of patients ranged between 16 and 70 years old (median 31 years old); 8 were males and were examined after a mean duration of 22 days of symptoms. Cortical venous thrombosis was attributed to puerperium in 4 patients, infection in 5 patients, and pregnancy, dehydration, insect bite, and migraine in 1 patient each. Superior sagittal sinus was involved in 15 patients, transverse sinus in 16 patients, straight sinus in 3 patients, sigmoid sinus in 7 patients, and cavernous and deep system in 1 patient each. A total of 12 patients had more than one sinus involvement. D-dimer was positive in 20 patients and correlated with the duration of symptoms but not with the extent of sinus thrombosis and the outcome. Conclusion: D-dimer is useful in patients suspected of CVST and patients with positive test results should be urgently sent for MR imaging.

Surgical considerations for 'intrinsic' brainstem gliomas: proposal of a modification in classification.

Abstract: Background: Brainstem gliomas are highly heterogeneous tumors both in their clinical manifestation and in their pathology. Despite significant advances in the surgery for brainstem gliomas many aspects of this pathology are still unclear Objective: To evaluate the clinical, radiological and surgical outcome of 40 focal 'intrinsic' brainstem gliomas and propose a surgical strategy-oriented classification. Materials and Methods: A total of 40 focal 'intrinsic' (expanding variety) tumors have been operated over a period of 8.5-years (January 1998-June 2007). Our criteria included patients with (1) well-defined gadolinium enhancing tumor; (2) relatively long duration of symptoms (> six months) and (3) good neurological functional status and independent for all activities of daily living. The cutoff size of 2 cm was not rigidly adhered to. Results: The 'intrinsic' brainstem tumors were classified into three types: Expanding, diffuse infiltrative and pure ventral varieties. Only patients with expanding variety of brainstem gliomas were subjected to surgery, mean age 19.2 years (range 4-55 years) and male to female ration mean: 3:2). The tumor location included pons (n=19), midbrain (n=13) and medulla (n=8). Surgical approaches included midline suboccipital (n=28), retromastoid (n=7), subtemporal (n=3) and supracerebellar-infratentorial (n=2). Thirty-two cases with 'diffuse infiltrative' and 'pure ventral' variety were given radiotherapy only. Histology pathology revealed pilocytic variety (n=10), Grade II (n=17) and Grade III (n=13). There was one death in the surgical series (due to aspiration). Complications included meningitis (n=2), wound infection (n=1), chest infection (n=5) and transient mutism (n=1). Follow-up ranged from 3-68 months. Overall, 36 improved /remained same and three worsened in their clinical status at the time of discharge. Conclusion: The surgical management of intrinsic brainstem tumors presents a surgical challenge; radical excision yielded a good outcome in the majority of cases. The authors propose a classification system for 'intrinsic' brainstem tumors for defining surgical strategy.

Efficacy of pulsed electromagnetic field therapy in healing of pressure ulcers: A randomized control trial.

Abstract: Background : Pressure ulcers are one of the most common complications in health care settings. Still there are no optimal protocols to manage the pressure ulcers. Aim : To assess the effectiveness of pulsed electromagnetic field therapy (PEMF) in healing of pressure ulcers in patients with neurological disorders. Design : Randomized double blind control trial. Setting : Neurological rehabilitation department in a university research hospital. Participants : Twelve patients (M:F, 9:3) having neurological disorders, with age between 12-50 years (mean 30.16611.32 yrs) and 24 pressure ulcers. Intervention : Six patients with 13 ulcers received PEMF therapy and the remaining 6 patients with 11 ulcers received sham treatment, for 30 sessions (45 minutes each) using the equipment 'Pulsatron'. The frequency of PEMF was set at 1 Hz with sine waves and current intensity of 30 mili ampere. Whole body exposure was given in both the groups. Outcome Measures : Bates-Jensen wound assessment tool (BJWAT) score was used as main outcome measure and scores at the end of session were compared with initial scores and analyzed. Similarly National Pressure Ulcer Advisory Panel (NPUAP) scores were compared and analyzed as secondary outcome measure. Results : Thirteen ulcers were in stage IV and 11 were in stage III at the start of the study. Significant healing of ulcers was noted, BJWAT scores, in both the treatment and sham groups (P < 0.001 and 0.003 respectively) at the completion of the study. However, when comparing between the groups, healing was not significant (P = 0.361). Similarly trend was noted with NPUAP scores with no significant difference between the treatment and sham groups (P = 0.649) at the completion of study. Conclusions : No significant difference in pressure ulcer healing was observed between PEMF treatment and sham group in this study.

The prevalence and clinical features of restless legs syndrome: A door to door population study in Orhangazi, Bursa in Turkey

Abstract: Background : Restless leg syndrome (RLS) has negative effects on the quality of life of the patients. Epidemiological studies on RLS are limited. Aim : To determine the prevalence of RLS among people aged 40 years and above in Orhangazi district of Bursa, Turkey. Materials and Methods : The study was carried out between 2004 and 2005. This population-based study was conducted in two phases. In the phase 1, residents conducted door-to-door interviews using a short questionnaire to determine the possible cases of RLS. In the phase 2, physicians ascertained the suspected cases of RLS. Diagnosis of RLS was made using the criteria proposed by the International Restless Legs Syndrome Study Group (IRLSSG) and also the rating scale for RLS. Results : Out of 1, 256 subjects screened in phase 1, 1,124 (89.6%) were included in the study. Also, 161 cases with suspected symptoms of RLS were detected in phase 1, 49 of the suspected cases could not be evaluated in phase 2. A diagnosis of RLS was also made in 60 cases during phase 2. Conclusions : We defined the prevalence of RLS as 9.71% among 40 years of age and older population. The prevalence of RLS did not differ by age and was 2.6 times more in women. None of the patients were diagnosed as RLS sought medical care for the symptoms, possibly due to lack of knowledge of patients and physicians about RLS. Prevalence studies help to provide knowledge on morbidity, which is essential for diagnosis and early treatment.

Methylenetetrahydrofolate reductase C677T polymorphism in Iraqi patients with ischemic stroke.

Abstract: Background: Data are conflicting concerning the association between ischemic stroke and methylenetetrahydrofolate reductase (MTHFR) C677T mutation. Studies addressing this matter in developing countries are limited. Aim: This study was undertaken to evaluate MTHFR C677T gene polymorphism as a possible risk factor in patients with ischemic stroke in Iraq. Settings and Design: A case-control study in a major teaching hospital in Northern Iraq. Materials and Methods: Study population included 70 patients with ischemic stroke diagnosed by computed tomography (CT) or magnetic resonance imaging (MRI) and 50 controls matched by age and sex. All the patients and controls had detailed neurologic examination and blood sugar, lipid profile, total homocysteine, as well as, MTHFR gene analysis. The MTHFR C677T mutation status was detected in the amplified products using reverse hybridization to specific mutant and wild oligonucleotide probes by a colorimetric microwell plate method. Statistical Analysis: Mann-Whitney U test and Chi-square tests were used to find the significance. Results: The median age of the patients was 60 years and 54% were males. The MTHFR C677T gene analysis detected TT genotype in 20% of patients and in 6% of controls and CC genotype in 37% of the patients and in 54% of the controls. The calculated risk of ischemic stroke in the subjects with TT genotype was 4.85 times more than the subjects with CC genotype (P=0.03). Serum homocysteine level was significantly higher in the patients than the controls (P=0.02). The serum homocysteine levels were significantly higher in those with TT and CT genotypes when compared to those with CC genotype (P < 0.001 and P=0.04, respectively). Conclusion: In the Iraq population studied MTHFR C677T TT genotype was a significant risk factor for ischemic stroke and it was related to the increased total homocysteine levels and the risk for ischemic stroke was graded with increasing MTHFR 677T allele dose.

Transverse myelitis spectrum disorders

Abstract: Acute transverse myelitis (ATM) is an inflammatory demyelinating disorder that affects the spinal cord focally resulting in motor sensory and autonomic dysfunction. Establishing the diagnosis of ATM is not as difficult as determining the possible etiology. There is a difference in the perception of ATM seen in the West as compared to developing countries. In the West multiple sclerosis (MS) is the most common inflammatory disorder of the central nervous system. An attack of ATM may be the beginning of MS. However, this may not be the case in developing countries where MS is uncommon. Most often transverse myelitis is monophasic and at best represents a site-restricted form of acute disseminated encephalomyelitis (ADEM). Traditionally the combination of optic neuritis and ATM, occurring as a monophasic illness would have been called as neuromyelitis optica (NMO). Changing concepts in the definition of NMO and the discovery of a biomarker, neuromyelitis optica immunoglobulin (NMO_IgG), has changed the way relapsing autoimmune disorders are being perceived currently. A variety of idiopathic inflammatory disorders such as Japanese form of optic spinal MS, recurrent myelitis, and recurrent optic neuritis have been brought under the umbrella of neuromyelitis spectrum disorders because of the association with NMO-IgG. Complete transverse myelitis accompanied by longitudinally extensive transverse myelitis which is seronegative for this biomarker has also been reported from several countries including Japan, Australia, and India. Thus, ATM is a heterogeneous disorder with a varied clinical spectrum, etiology, and outcome.

Association of meningioma and intracranial aneurysm: Report of five cases and review of literature

Abstract: Meningiomas associated with intracranial aneurysms are very rare. The co-existence of both lesions is not only a diagnostic challenge but also has important therapeutic implications. We analyzed our experience of five such patients, the second largest group in the literature. All the lesions were successfully managed surgically. All the patients were female. The location of the meningioma was clinoidal (2), planum sphenoidale (1), petroclival (1) and pterional (1). Three patients had posterior communicating (PCOM) artery aneurysm associated with meningioma. The other aneurysms were internal carotid artery (ICA) and anterior communicating artery (ACOM). One patient with pterional meningioma had an anomalous meningeal artery arising from the right ophthalmic artery. In three patients both lesions underwent treatment at the same sitting. In two patients meningioma underwent excision first followed by aneurysm clipping. From our experience the incidence of meningiomas associated with aneurysms was 1.1%, which is not higher than the incidence of aneurysms in the general population.

Epilepsy surgery in India

Abstract: This review traces the evolution of epilepsy surgery in India from the beginning to the present state. During the last one and half decades, surgical treatment of epilepsies has made resurgence in this country and at present a few centers have very active and sustained epilepsy surgery programs. Within a 14-year period, the R. Madhavan Nayar Center for Comprehensive Epilepsy Care, Trivandrum, has undertaken over 1000 epilepsy surgeries. However, in the whole country, annually, not more than 200 epilepsy surgeries are currently being performed. This number is a miniscule when compared to the number of potential surgical candidates among the vast population of India. The enormous surgical treatment gap can only be minimized by developing many more epilepsy surgery centers in different parts of our country.

Current treatment strategy in the management of vestibular schwannoma.

Abstract: Background: The changing trends in the management of vestibular schwannoma (VS) in our practice over the last two decades as well as the current status are presented here. Materials and Methods: The observations are based on the experience of 559 consecutive cases of VS operated by the first author between 1987 and 2008, 438 of which were operated by microsurgery and 139 by gamma knife radiosurgery (GKR) (18 of which were previously operated by the authors). A detailed analysis of microsurgically managed patients in two different periods (100 consecutive patients each before 1993 and 2008) were compared to see the changing trend and document current results. Results and Discussion: In the initial experience (1990s), the emphasis in microsurgery was preserving life, total excision of tumor and preservation of function in that order. In the 21 st century, the emphasis in microsurgery has been all about functional preservation. In 100 consecutive cases of VS (excluding neurofibromatosis-2) that were treated microsurgically between 2005-08, there were four small tumors (<2 cm), 14 medium-sized tumors (2-3 cm) and 82 large tumors (≥3 cm). The total excision rate was 83%. The facial nerve anatomical preservation rate was 96% and function was Grade III House-Brackmann (HB) or better in 87%. Both the total excision rate and facial function of Grade II HB or better were 100% in cases with tumor size less than three cm. Functional hearing preservation was achieved in ten cases. There was no operative mortality. Conclusion: Total excision of VS, though aimed at, is no more pursued at the cost of facial function. Moreover, microsurgery, radiosurgery and observation are all valid options in the management of VS and choosing the correct modality helps in achieving optimal outcome.

Duchenne muscular dystrophy: A clinical, histopathological and genetic study at a neurology tertiary care center in southern India

Abstract: Background : Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy that affects young boys and the dystrophin gene on the X chromosome has been found to be associated with the disorder. Materials and Methods : In this prospective study, 112 clinically diagnosed DMD patients had muscle biopsy and were tested for exon deletions. Genotyping was also carried out at STR44, STR45, STR49 and STR 50 markers in 15 families. Results : Of the 112 clinically suspected DMD patients, the diagnosis of DMD was confirmed by histopathology and/or genetics in 101 patients. The mean age of onset was 3.1±1.44 years (1-6 years) and the mean age at presentation was 8.0±3.1 years (1.1-18.0 years). Delayed motor milestones were present in 63 (62.3%) patients. The mean creatine kinase value was 11822.64±8206.90 U/L (1240-57,700). Eighty-four patients had muscle biopsy and immunohistochemistry was done in 60 muscle samples, all of which demonstrated absence of dystrophin staining. Of the 60 dystrophin-negative cases, 73% showed deletion of at least one exon. Single exon deletion was found in 20.4%. Distal hotspot Exons 45, 47, 49 and 50 were the commonly deleted xenons and the deletion rates were 36%, 35%, 33.7% and 38.5% respectively. Conclusions : In this study population in south India the deletion rate was 73% and were more frequent in the distal end exon. With the availability of genetic analysis, the first investigation of choice in DMD should be genetic studies and muscle biopsy should be considered only if the genetic tests are negative or not available.

Polymorphism of the methylenetetrahydrofolate reductase gene association with homocysteine and ischemic stroke in type 2 diabetes.

Abstract: Background : Ischemic stroke is a frequent heterogeneous multifactorial disease. A number of genetic mutations and environmental factors have been implicated. A polymorphism in the gene for methylenetetrahydrofolate reductase (MTHFR) has been reported to be associated with hyperhomocysteinemia a risk for atherosclerotic vascular diseases. Aim : A cross-sectional study was performed to determine the relationship between the gene polymorphism for MTHFR and ischemic stroke in type 2 diabetes mellitus. Materials and Methods : Of the 215 unrelated patients with type 2 diabetes mellitus recruited, 119 patients had ischemic stroke, Control group included 142 healthy subjects. The genotype of the subjects for the C677T polymorphism of MTHFR was analyzed by using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) followed by HinfI digestion. Plasma total homocysteine (Hcy) levels were measured using high-performance liquid chromatography (HPLC) with fluorescence detection. Results : The genotype distribution did not differ between the control subjects and type 2 diabetic patients (P > 0.05). Plasma homocysteine levels were markedly higher in diabetic patients with TT genotype than those with CC or CT genotype (P > 0.05). Ischemic stroke was more frequently observed in type 2 diabetic patients with the TT genotype than in those with the CT and CC genotype (odds ratio=4.04, 95% CI=1.95-8.34, P=0.0036). Logistic regression analysis revealed that the C677T mutation of MTHFR gene was independently associated with ischemic stroke in type 2 diabetes. Conclusion : MTHFR C677T gene polymorphism associated with a predisposition to hyperhomocysteinemia could constitute a useful predictive marker for ischemic stroke in type 2 diabetic Chinese patients.

Experience of microsurgical treatment of brainstem cavernomas: report of 37 cases.

Abstract: Background: Cavernomas are rare in the brainstem and account for 18-35% of central nervous cavernomas and can cause recurrent hemorrhages, devastating neurological deficits and mortality. Objectives: To summarize the experience of microsurgical treatment of brainstem cavernomas and to investigate curative effect of microsurgical treatment of brainstem cavernomas. Materials and Methods: A retrospective analysis clinical data of 37 patients with brainstem cavernomas seen between 2003 and 2007. The analysis included age distribution, hemorrhage rates, clinical presentation, location of the lesions, and preoperative and postoperative Karnofsky Performance Scale (KPS) scores. The surgical indications, the timing of surgery and the surgical techniques were also assessed. Results: All the 37 patients received microsurgical resections, there was no surgery-related mortality. Histopathological examination confirmed the diagnosis of cavernoma. Postoperatively, 20 patients had functional improvement, 15 patients had no change in the neurological status, and two patients deteriorated. Early surgery was associated with better outcomes. Mean followed up period was 21.5 months (range 6-36 months). During the follow-up 20 patients had resumed activities of daily living (KPS scores of 90-100), 10 patients were able to self-care with some efforts (KPS scores of 70-80), five patients needed considerable assistance (KPS score of 50-60) and two patients suffered hemiparesis (KPS scores of 40). None of the patient had recurrent hemorrhage. Conclusions: Brainstem cavernomas can safely be resected. Successful resection of brainstem cavernomas can be achived by optimal surgical approaches, feasible entry zone and meticulous microsurgical techniques. The goal of surgical intervention should be the total resection of the lesion without any deteriorative in the neurological deficits.

Spontaneous intracranial extradural hematoma: case report and literature review.

Abstract: Spontaneous extradural hematoma (EDH) is an uncommon form of intracranial hematoma and is caused by the adjacent sinus and otic infections, dural vascular malformations and disorders of blood coagulation. We report spontaneous EDH in a 54-year old women with chronic kidney disease (CKD) on intermittent hemodialysis. She was successfully managed by conservative treatment. The EDH in her was probably related to the coagulation abnormalities associated with CKD, hemodialysis or heparin use during the dialysis.

Comprehensive rural epilepsy surveillance programme in Uttarakhand state of India

Abstract: of hyperintensity extending along corticospinal tracts as well as involving corpus callosum [Figure 1]. This peculiar imaging appearance has hitherto not been reported in a classical case of ALS. T2-weighted image showing high signal intensity involving the corticospinal tract extending into the anterolateral column of the spinal cord has been described.[1] Involvement of the corpus callosum has also been reported.[2] In addition, T2weighted MRI typically demonstrates low signal intensity in the motor cortex; this finding has been attributed to T2 shortening due to iron deposition.[1] A lesion distribution simulating a garland has been described with Alexander disease, metastatic colorectal adenocarcinoma, and acute postinfectious glomerulonephritis.[3-5]