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Acute disseminated encephalomyelitis (ADEM) presents as an acute-onset neurological dysfunction following a triggering event such as an infection or vaccination. Patients present with polysymptomatic neurological dysfunction, and imaging shows multifocal white matter lesions in the brain and spinal cord. Clinical evaluation, magnetic resonance imaging, and cerebrospinal fluid study are most useful in establishing the diagnosis and ruling out important differential diagnoses. Corticosteroids are the mainstay of treatment and the role of other modalities of treatment, such as plasma exchange and intravenous immunoglobulin, require further study. Prognosis is generally good. The recently proposed consensus definitions are likely to facilitate delineation of ADEM from other acquired demyelinating disorders.

We report MR imaging features of vanishing white matter disease in a 7-year-old boy, who manifested with seizures, aphasia, spastic quadriparesis and myoclonic jerks. MRI of brain showed diffuse white matter signal changes of CSF intensity in all the sequences. MR spectroscopy of white matter showed severe decrease in NAA, choline and creatine and presence of lactate peak. Additional notable findings were diffuse extensive brain stem and thalamic atrophy. The clinico-radiological correlation was consistent with the diagnosis of vanishing white matter disease. Reporting of such cases may widen the spectra of these disorders.

Aims: Urodynamics (UDS) is often indicated for multiple sclerosis (MS) patients either at presentation to specialized medical centers or after failure of conservative management of lower urinary tract dysfunction (LUTD). However, the ideal moment and context to indicate this exam in this group of patients remain controversial. We aimed to establish a consensus panel to address the role of UDS in MS patients. Methods: A panel representing urology, rehabilitation medicine, and neurology skilled in neuro‐urology participated in a consensus‐forming project using a Delphi method to reach consensus on the role of UDS in MS patients. Results: In total, five experts participated. All panelists participated in the four phases of the consensus process. Consensus was reached if ≥70% of the experts agreed on recommendations. To facilitate a common understanding among all experts, a face‐to‐face consensus meeting was held in Florence in September 2017 and then with a follow‐up teleconference in March 2018. By the end of the Delphi process, formal consensus was achieved for 100% of the items and an algorithm was then developed in a face‐to‐face meeting in Philadelphia in August 2018. The final expert opinion recommendations were approved by the unanimous consensus of the panel. Conclusions: UDS represents an important diagnostic tool for MS patients and is particularly useful to evaluate the pattern of LUT dysfunction in high‐risk patients. There is a lack of high‐evidence level studies to support an optimal urodynamic long‐term follow‐up protocol. [ABSTRACT FROM AUTHOR]

Byline: J. Panicker, S. Sinha, A. Taly, A. Mahadevan, C. Sagar, S. Srikanth, G. Arunodaya, S. Shankar A 37-year-old gentleman presented with macrocephaly since early childhood and progressive impairment of [...]

Byline: S. Ravishankar, S. Sinha, A. Taly, J. Panicker We report MR imaging features of vanishing white matter disease in a 7-year-old boy, who manifested with seizures, aphasia, spastic quadriparesis [...]

Byline: J. Panicker, S. Sinha, A. Taly, S. Ravishankar, G. Arunodaya A 19-year-old gentleman presented with slowly progressive spastic paraparesis, 2 years after the therapeutic lienorenal shunt for portal hypertension [...]

Byline: J. Panicker, D. Nagaraja, E. Ratnavalli, P. Pal Sir, Paraneoplastic peripheral neuropathy is a well-described entity.[1],[2] Amongst the various manifestations, mononeuritis multiplex is relatively unusual and is usually associated [...]

Byline: J. Panicker, M. Thomas, K. Pavithran, M. Nair, P. Sarma Sir, We appreciate the observations made regarding our article Morbidity predictors in ischemic stroke. Neurology India 2003;51:49-51. The analysis [...]

Background and purpose: Bowel symptoms are well documented in mitochondrial disease. However, data concerning other pelvic organs is limited. A large case–control study has therefore been undertaken to determine the presence of lower urinary tract symptoms (LUTS) and sexual dysfunction in adults with genetically confirmed mitochondrial disease. Methods: Adults with genetically confirmed mitochondrial disease and control subjects were recruited from a specialist mitochondrial clinic. The presence and severity of LUTS and their impact on quality of life, in addition to sexual dysfunction and bowel symptoms, were captured using four validated questionnaires. Subgroup analysis was undertaken in patients harbouring the m.3243A>G MT‐TL1 mitochondrial DNA mutation. A subset of patients underwent urodynamic studies to further characterize their LUTS. Results: Data from 58 patients and 19 controls (gender and age matched) were collected. Adults with mitochondrial disease had significantly more overactive bladder (81.5% vs. 56.3%, P = 0.039) and low stream (34.5% vs. 5.3%, P = 0.013) urinary symptoms than controls. Urodynamic studies in 10 patients confirmed that bladder storage symptoms predominate. Despite high rates of LUTS, none of the patient group was receiving treatment. Female patients and those harbouring the m.3243A>G MT‐TL1 mutation experienced significantly more sexual dysfunction than controls (53.1% vs. 11.1%, P = 0.026, and 66.7% vs. 26.3%, P = 0.011, respectively). Conclusions: Lower urinary tract symptoms are common but undertreated in adult mitochondrial disease, and female patients and those harbouring the m.3243A>G MT‐TL1 mutation experience sexual dysfunction. Given their impact on quality of life, screening for and treating LUTS and sexual dysfunction in adults with mitochondrial disease are strongly recommended. [ABSTRACT FROM AUTHOR]

The article presents a case of a 55-year-old female who has a history of hearing difficulty without tinnitus, vertigo or aural fullness to discuss her unexpected diagnosis of nystagmus, unequivocal gait, and truncal ataxia.

Introduction: The surgical trans epicondylar axis (sTEA) is considered the gold standard for optimum rotation of the femoral component; however, no consensus exists on tibial component positioning. The objectives of this study were to determine the relationship of sTEA to various femoral and tibial reference axes in varus osteoarthritis (OA) knees and (ii) to study the intra-observer and inter-observer variability of the axis relationships. Materials and methods: The study was done on preoperative computerised tomogram (CT) scans of 110 varus knees to assess the rotational relationships respectively of femoral side sTEA with whitesides line (WSL), posterior condylar axis (PCA), clinical trans epicondylar axis (cTEA) and on the tibial side sTEA with posterior tibial margin (PTM), anterior condylar axis (ACA), Akagi’s line and line from the geometric centre of the tibial plateau to 1/3rd tibial tubercle (line GC 1/3rd TT). Results: On the femoral side the mean angles of sTEA with WSL, PCA, cTEA were 95.64° ± 2.85°, 1.77° ± 1.88°, 4.19° ± 0.99° respectively. On the tibial side, the mean angles of sTEA with, PTM, ACA, Akagi’s line, and line GC 1/3rd TT were 1.10° ± 4.69°, 11.98° ± 4.51°, 2.43° ± 4.35°, 16.04° ± 5.93° respectively. Conclusion: Contrary to the generalization, TEA has variable relationships. The surgical trans epicondylar axis was not at the assumed 3° of external rotation to PCA in 85% of knees, nor perpendicular to WSL in >95% of knees. Of the four tibial axes, Akagi’s line was the least variable with sTEA. Furthermore, surgeons should also be aware of the multiple reference axes and the range of deviation from sTEA to optimize the rotational alignment of components.

Abstract: Massive blood transfusion is replacement of a patient’s total blood volume in less than 24h, or acute administration of more than half the patient’s estimated blood volume per hour. This case report describes the clinical profile, laboratory parameters and management of a post operative patient who lost nearly double her blood volume in 36h. A 27yr old Indian woman with no history of any medical illness, or co-morbidities underwent elective cesarean section for placenta praevia totalis. The maximal allowable blood loss calculated for her weight with target haemoglobin of 10g% was 450ml. Though adequate haemostasis was achieved before closure, she had significantly increased bleeding PV 1h post-operatively. Over the next 24h she underwent a subtotal and then a total hysterectomy. Blood loss within this period amounted to 6500ml. The following day she lost 1900ml blood through the drains. As is the approach to a surgical patient with massive blood loss, it was ensured that the surgical cause of bleeding is controlled with simultaneous blood component therapy so as to maintain haemostasis, blood oxygen carrying capacity, oncotic pressure and plasma biochemistry. The patient received a total of 18 units Packed Red Blood Cells, 20 units Fresh Frozen Plasma and six platelets over 36h. The patient showed good recovery. Key points in therapy include control and management of underlying cause, supportive treatment with appropriate blood components for maintaining tissue perfusion and oxygenation. [Copyright &y& Elsevier]

Background Lower urinary tract dysfunction (LUTD) in multiple sclerosis has been well documented. However, its occurrence and outcome in acute disseminated encephalomyelitis (ADEM) has only been variably reported. Objective To evaluate LUTD in ADEM, correlation with other neurological deficits, and outcome. Methods Patients with ADEM having significant LUTD were evaluated. LUTD was evaluated by symptom analysis, ultrasonography, and urodynamics. Storage symptoms were managed using antimuscarinics and significant voiding dysfunction by catheterization. Results Of 61 patients, 20 (33%) had LUTD. Voiding dysfunction was more common and 16 patients were in urinary retention. Cystometry demonstrated detrusor overactivity in four patients and underactivity in four patients. Incontinence was reported more often in patients with frontoparietal white matter changes in MR imaging. LUTD was found to be associated with occurrence of paraparesis or tetraparesis, though did not predict functional outcome at discharge. At 3 months follow up, five patients continued to have LUTD and urgency and hesitancy were commonest symptoms. Conclusion LUTD is common in ADEM, especially in patients with lower limb pyramidal involvement and its causes multifactorial. Presence of LUTD does not influence the functional outcome of patients with ADEM. Recovery may be incomplete and symptoms may persist even after recovery of other neurological deficits. [ABSTRACT FROM AUTHOR]

Bladder symptoms in multiple sclerosis (MS) are common and distressing but also highly amenable to treatment. A meeting of stakeholders involved in patients' continence care, including neurologists, urologists, primary care, MS nurses and nursing groups was recently convened to formulate a UK consensus for management. National Institute for Health and Clinical Excellence (NICE) criteria were used for producing recommendations based on a review of the literature and expert opinion. It was agreed that in the majority of cases, successful management could be based on a simple algorithm which includes using reagent sticks to test for urine infection and measurement of the post micturition residual urine volume. This is in contrast with published guidelines from other countries which recommend cystometry. Throughout the course of their disease, patients should be offered appropriate management options for treatment of incontinence, the mainstay of which is antimuscarinic medications, in combination, if necessary, with clean intermittent self-catheterisation. The evidence for other measures, including physiotherapy, alternative strategies aimed at improving bladder emptying, other medications and detrusor injections of botulinum toxin A was reviewed. The management of urinary tract infections as well as the bladder problems as part of severe disability were discussed and recommendations agreed. [ABSTRACT FROM AUTHOR]