Management of patients with Reflex Vasovagal syncope with a protocol involving a yoga maneuver Tadasana.

BACKGROUND: There is no universally followed protocol for managing Reflex Vasovagal syncope (VVS). METHODS: VVS patients were treated with a 2 step protocol. Step I - counseling, hydration, physiotherapy, and Tadasana Yoga maneuver. Patients with ≥2 VVS recurrences were given step II care - intensification of step I, elastic stockings,and pharmacotherapy. Follow-up included assessment by periodic functional status questionnaires. RESULTS: 157 patients (103 males & 54 females,mean age - 53 ± 20 years & mean LVEF - 62 ± 5%.) experienced 867 total events - 382 syncopal, and 485 near syncopal episodes over 14 ± 9 months. After step I protocol, the mean total, syncopal and near syncopal events declined from 5 ± 7 to 0.3 ± 1.2 (P < 0.0001), 3 ± 2 to 0.1 ± 0.4 (P < 0.0001) and 3 ± 6 to 0.2 ± 1.1 (P < 0.0001) respectively. Twenty (12.7%) patients had 53 event recurrences, 15- syncopal episodes in 7 and 38 near syncope events in 13. After step II, 5 patients had 14 events. At 33 ± 15 months, in 152 patients (96.8%) there were no recurrences and syncope was prevented in all (100%). The median total, syncopal and near syncopal events declined from 3 to 0,(p < 0.001) 2 to 0 (p < 0.001) and 1 to 0 (p < 0.001) respectively. There was an improvement in all the 3 quality of life parameters. CONCLUSION: We demonstrate a simple and effective protocol that can be universally adopted to prevent VVS recurrences,with improvement in quality of life.

Aetiology and outcome of generalized convulsive status epilepticus in elderly.

PURPOSE: Convulsive status epilepticus (CSE) is a common neurologic emergency in elderly people. The current study elaborates the clinical characteristics and outcome of CSE in elderly patients. METHODS: Analysis of data of generalized CSE patients, aged 60 years and above admitted at the neurointensive care unit (NICU) was performed. The primary outcome for analysis was in-hospital mortality. The study population was divided into groups based on progression of CSE and mortality to analyze difference in study variables. Mortality of the group was analyzed using life tables. RESULTS: A total of 33 patients satisfied the inclusion criteria from medical records of 212 patients with CSE. Mean age of the study population was 67.0 ± 6.8 years; 69.7% were men. Acute symptomatic aetiology was the commonest cause of CSE (60.6%); nine (27.3%) patients progressed to refractory status epilepticus (RSE) of which five patients had prolonged RSE. The overall mortality was 18.2%. Complications of mechanical ventilation and mean age were higher in patients who died. Though vascular aetiology was the leading cause of CSE (39.3%), it was not associated with progression to RSE or mortality. Acute symptomatic aetiology accounted for five out of the six deaths in the entire cohort. CONCLUSION: Less than one-third of elderly patients with CSE progressed to RSE. Vascular aetiology, the leading cause of generalized CSE in elderly, was not associated with progression to RSE and mortality. Acute symptomatic aetiology was associated with high mortality.

Impact of family support on psychiatric disorders and seizure control in patients with juvenile myoclonic epilepsy.

OBJECTIVE: Psychiatric disorders (PDs) are frequently observed in patients with juvenile myoclonic epilepsy (JME). In this study, we aimed to assess factors associated with PDs in patients with JME. METHODS: Retrospective analysis of data of 90 consecutive patients with JME was performed. Assessment of DSM-IV Axis I clinical disorders was done using Structured Clinical Interview for Axis I. Diagnosis of PDs is made when the score exceeds the threshold provided by the DSM-IV. We also applied the Global Assessment of Functioning (GAF) scale which is part of the multiaxial evaluation of the DSM-IV (Axis-V). Using seizure frequency score at presentation, we classified subjects into controlled and uncontrolled groups. RESULTS: In the current cohort, 29 (32.2%) patients were diagnosed with PDs. Fewer patients with PDs had family support (48.3% vs. 83.6%; p=0.001). Lifetime prevalence of PDs was higher among patients with current PDs (96.6% vs. 18.0%; p<0.0001). Subthreshold illness was not different between the groups (17.2% vs. 27.9%; p=0.204). Mean GAF was higher in patients without PDs than in patients with PDs (89.19±6.92 vs. 64.22±9.76; p<0.0001). Patients with PDs had lower seizure control (7.8% vs. 73.1%; p<0.0001) compared with patients without PDs. Logistic regression analysis for factors associated with diagnosis of PDs revealed that none of the factors significantly affected the odds of seizure control. Patients with lack of family support had poor seizure control (0% vs. 36.9%; p<0.0001); 51.7% of patients with JME with PDs reported lack of family support. Patients with family support had lower lifetime prevalence of PDs (30.8% vs. 76.0%; p<0.0001), whereas patients with JME without family support had lower levels of education (8.0% vs. 35.4%; p=0.009). CONCLUSION: Lack of family support is associated with poor seizure control and higher incidence of PDs in patients with JME. Lack of family support increases neither the odds of PDs nor seizure control.

Twelfth cranial nerve involvement in Guillian Barre syndrome.

Guillian Barre Syndrome (GBS) is associated with cranial nerve involvement. Commonest cranial nerves involved were the facial and bulbar (IXth and Xth). Involvement of twelfth cranial nerve is rare in GBS. We present a case of GBS in a thirteen years old boy who developed severe tongue weakness and wasting at two weeks after the onset of GBS. The wasting and weakness of tongue improved at three months of follow up. Brief review of the literature about XIIth cranial nerve involvement in GBS is discussed.

Diagnosis of mitochondrial diseases: clinical and histological study of sixty patients with ragged red fibers.

BACKGROUND: Mitochondrial diseases are caused by mutations in mitochondrial or nuclear genes, or both and most patients do not present with easily recognizable disorders. The characteristic morphologic change in muscle biopsy, ragged-red fibers (RRFs) provides an important clue to the diagnosis. MATERIALS AND METHODS: Demographic data, presenting symptoms, neurological features, and investigative findings in 60 patients with ragged-red fibers (RRFs) on muscle biopsy, seen between January 1990 and December 2002, were analyzed. The authors applied the modified respiratory chain (RC) diagnostic criteria retrospectively to determine the number of cases fulfilling the diagnostic criteria of mitochondrial disease. RESULTS: The most common clinical syndrome associated with RRFs on muscle biopsy was progressive external ophthalmoplegia (PEO) with or without other signs, in 38 (63%) patients. Twenty-six patients (43%) had only external ophthalmoplegia, 5 (8%) patients presented with encephalomyopathy. Specific syndromes were the presenting feature in 8 (13%), Kearns-Sayre syndrome (KSS) in 4 and myoclonus epilepsy with ragged-red fibers (MERRF) in 4. Myopathy was the presenting feature in 5 (8%) and 4 presented with infantile myopathy. Of the 60 patients, 18 (30%) had proximal muscle weakness. Two patients with KSS and one patient with myopathy had complete heart block necessitating pace making. When the modified RC diagnostic criteria were applied, only 26 (43%) patients had one other major criterion in addition to RRFs for the diagnosis of mitochondrial diseases. The remaining 34 (57%) patients with RRFs on muscle biopsy had only some clinical features suggestive of RC disorder but did not fulfill the clinical criteria (of the modified diagnostic criteria) for the diagnosis of mitochondrial diseases. CONCLUSION: In patients with clinical features suggestive of RC disorder, demonstration of RRFs on muscle biopsy helps in confirming the diagnosis of mitochondrial disease in only a subgroup of patients.