Neuromyelitis Optica Spectrum Disorders in North Indian Population: Experience from a Tertiary Care Center.

Introduction: To understand neuromyelitis optica spectrum disorders (NMOSDs) better we need to study them in different populations. This prospective study was conducted to characterize clinical, serological, radiological, and therapeutic profile of NMOSDs in a North Indian population. Materials and Methods: This study included 81 patients with NMOSDs. All patients underwent detailed history and examinations and were followed at 3 monthly intervals. They were evaluated using standard investigations including gadolinium-enhanced magnetic resonance imaging (MRI) of the brain and spine with thin section optic nerve cuts and treated as per the standard guidelines. Data were recorded meticulously. Results: The mean age was 33.7 ± 13.4 years. The mean age at disease onset was 31.2 ± 13.5 years. Female-to-male ratio was 1.9:1. About 32.1% of patients presented with optic neuritis (ON), 56.8% with transverse myelitis (TM), and 11.1% with both ON and TM. The mean time from disease onset to diagnosis was 16.17 ± 23.09 months. Muscle atrophy, Lhermitte symptom, and tonic spasms were common. Foster-Kennedy syndrome-like presentation was seen in 8.6%. NMO antibodies were positive in 41 patients. MRI revealed involvement of <4 vertebral segments in 16.4% of patients with TM. Patients were managed as per standard guidelines. The mean follow-up duration was 15.3 ± 6 months. Approximately 88.9% had good functional outcome. Conclusion: NMOSDs are a common cause of demyelinating illnesses in Northern India. The response to treatment is excellent and most patients recover without residual disability.

Myoedema with pseudohypertrophic hypothyroid myopathy (Hoffman's syndrome).

Myoedema is an under-recognised neurological sign that can help the bedside diagnosis of metabolic or endocrine myopathies. Myoedema together with pseudo-hypertrophy make a likely diagnosis of hypothyroid myopathy, and its identification may avoid unnecessary investigations.

Magnetic resonance imaging (MRI) versus computed tomographic scan (CT scan) of brain in evaluation of suspected cavernous sinus syndrome.

BACKGROUND AND PURPOSE: The cavernous sinus is a unique region owing to anatomical factors and the pathologies affecting it. The diagnosis of cavernous sinus syndrome (CSS) predominantly relies on clinicoradiological correlation. We studied the utility of computed tomographic (CT) scan versus magnetic resonance imaging (MRI) in the diagnosis of CSS. METHODS: A prospective observational study was conducted in a tertiary care center in north India. All patients presenting with a clinical syndrome of cavernous sinus involvement with radiologically confirmed lesions were enrolled in the study. MRI and CT scan with cavernous sinus cuts were done and reviewed by experienced neuroradiologists for cavernous sinus lesions and compared with the final diagnosis. Sensitivity and specificity were calculated. RESULTS: We included 48 patients in our study. A final diagnosis was achieved in 41 out of 48 (85.6%) patients. Fungal infections (16 (33.3%)) constituted the commonest cause of CSS, followed by neoplastic involvement (13 (27.1%)) and Tolosa-Hunt syndrome (12 (25%)). Vascular involvement was seen in three (6.3%) patients. Other rare causes were seen in four (8.3%) patients. CT scan had an overall sensitivity of 14.6% in achieving a final diagnosis, whereas MRI had an overall sensitivity of 70.7%, with a statistically significant difference (p < 0.001). CONCLUSIONS: Although CT scan is a relatively cheap and accessible resource, its role in CSS diagnosis and management is limited because of poor yield. Hence, it is prudent to do an MRI as an initial investigation in cases of CSS.

Presence of allele CYP3A4*16 does not have any bearing on carbamazepine-induced adverse drug reactions in North Indian people with epilepsy.

OBJECTIVES: The objectives of this study were to determine the relationship between genetic polymorphisms in gene encodings for CYP3A4 and carbamazepine (CBZ)-induced dose-related side effects in North Indian people with epilepsy. PATIENTS AND METHODS: The current prospective study included 37 patients with CBZ-induced dose-related side effects and 102 patients who did not experience side effects while on CBZ. The genotyping for CYP3A4 allele (CYP3A4*16) was done using real-time polymerase chain reaction (RT-PCR) in Applied Biosystems 7500 RT-PCR System (USA). CBZ was administered in all patients at a dose varying from 15 to 20 mg/kg daily. RESULTS: Various demographic variables were comparable between the groups except that control of seizures was far better in controls. After testing, it was found that none of our patients had the presence of CYP3A4*16 allele. CONCLUSION: CYP3A4*16 allele is not represented significantly in North Indian people with CBZ-induced dose-related side effects.

Corrigendum to "Poor Functional Outcome in Patients with Voluntary Knee Instability after Anterior Cruciate Ligament Reconstruction".

[This corrects the article on p. 312 in vol. 12, PMID: 32903976.].

Poor Functional Outcome in Patients with Voluntary Knee Instability after Anterior Cruciate Ligament Reconstruction.

BACKGROUD: Anterior cruciate ligament reconstruction (ACLR) remains the gold standard treatment for anterior cruciate ligament (ACL) injury. However, a good functional outcome even after a successful surgery depends on multiple factors. It has been observed that certain patients with a chronic ACL injury demonstrate knee instability voluntarily. The authors observed that these patients might not perform well even after a successful surgery. This study aims to assess the outcome after ACL and other ligament reconstruction in patients with voluntary knee instability. METHODS: From a total of 824 patients who underwent ACLR, 13 patients with a history of voluntary knee instability were selected, and data of these patients (demographic and clinical profile) were obtained. Outcomes of surgery in this group of patients were evaluated by using Lysholm score and Tegner activity level. RESULTS: All patients were young men with a chronic ACL injury and manifested instability. Associated injuries were lateral meniscus tear in 3 patients, medial meniscus tear in 2, and posterolateral corner (PLC) injury in 3. ACLR was done using the semitendinosus-gracilis graft in all patients. Further, anterolateral ligament reconstruction was done in 2 patients and PLC reconstruction, in 3 patients. The mean Lysholm score was 54.76 (range, 48-62) preoperatively and 60.92 (range, 54-78) at a mean follow-up of 14.3 months (range, 11-26 months). The median Tegner activity level was 6 (range, 5-7) before injury and 4 (range, 3-5) at the final follow-up. Twelve of the 13 patients were able to demonstrate instability voluntarily at the time of the final follow-up. CONCLUSIONS: In patients with ACL and other ligament injuries who demonstrated voluntary knee instability, the functional outcome even after successful ligament reconstruction was poor.

Use of rituximab in muscle-specific tyrosine kinase antibody-positive myasthenia gravis: Preliminary observations from a tertiary care center in Northern India.

OBJECTIVES: Approximately 10%-15% of patients with myasthenia gravis (MG) are refractory to standard treatment. A sizable chunk of these patients is due to muscle-specific tyrosine kinase (MuSK) antibody-positive MG which often runs a severe course with frequent relapses and poor response to conventional treatment. We report six patients with refractory MuSK-positive MG who responded well to the treatment with rituximab. PATIENTS AND METHODS: In this prospective institute-based observational study, we report six MuSK antibody-positive MG patients, who did not achieve remission with standard treatment and were later started on rituximab infusion. RESULTS: There was a significant clinical improvement in all patients after starting rituximab. CONCLUSION: Rituximab is an effective immunomodulatory therapy in MuSK antibody-positive MG patients who are not responding to the standard treatment.

Similar functional outcome using single anterior portal and standard two portals technique in recurrent dislocation of shoulder.

PURPOSE: Recurrent dislocation of shoulder (RDS) is a common injury in high demand professionals, like athletes and military personnel. The treatment for the patients with Bankart lesion is the arthroscopic repair. This present study compares the outcomes of two different techniques of arthroscopic Bankart repair i.e. a standard two anterior portals technique and a single anterior portal technique in patients with RDS. METHODS: Patients with traumatic RDS met the inclusion criteria were managed with Bankart repair using either two anterior portals (Group A) or a single anterior portal (Group B) technique. Patients were evaluated before the intervention and at the mean follow-up of approximately two years using Rowe score, Oxford shoulder score and Tegner activity scale. RESULTS: The mean age of the patients in Groups A (n = 34) and B (n = 37) was 29.64 years and 29.05 years respectively (p = 0.66). The dominant shoulder was involved in 27 patients in Group A and 22 patients in Group B (p = 0.069). The operative time in Group A and B was 68.52 min and 46.35 min, respectively (p < 0.001). The complications at follow-up, the mean Rowe score and Oxford score improved significantly in both groups compared with the pre-operative values. However, the final outcome scores were not significantly different between the both groups. The median Tegner's score preoperatively and at follow-up was 7 and 6, respectively in Groups A and B. CONCLUSIONS: Single anterior portal technique is an effective treatment modality, yielding a similar outcome as two anterior portals technique in the management of RDS.

Neuromyelitis optica spectrum disorder and adenocarcinoma of ovary: a novel association.

Paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) is a rare clinical entity with less than 40 cases described in literature until today. Paraneoplastic NMOSD in association with adenocarcinoma of ovary has not been described yet. We present a case of paraneoplastic NMOSD in association with carcinoma ovary which improved following appropriate immunomodulation with pulse intravenous methylprednisolone and azathioprine.

Tuberculomas in "Critical" Locations of the Visual Pathway-A Masquerader.

Tuberculosis never stops fascinating physicians. The burden of tuberculosis is a major cause of disability. While drugs are often blamed when patients develop new focal deficits on treatment, the deterioration in these patients can have a myriad of causes including the development of new tuberculomas. Recognizing the critically located tuberculomas as potential causes of vision loss is indispensable. Here, we report two patients with strategic tuberculomas leading to vision loss. In one patient, the cause of visual morbidity was compression of the optic pathways; in the other patient, cortical vision loss was noted due to strategic location of tuberculomas.