Two women in their 60's are presented to us with sudden falls of acute onset. Prolonged observation revealed a gradually evolving syndrome of paroxysmal right sided faciobrachial dystonic (FBD) posturing lasting seconds. Both patients went on to develop hyponatremia, following which the episodes worsened and appeared on both sides. In both cases, prolonged electroencephalography monitoring and magnetic resonance imaging brain were normal and the response to conventional anticonvulsants was poor. One patient improved spontaneously over 6 months. The 2nd patient developed an amnestic syndrome and was started on intravenous methylprednisolone with which her movement disorder abated. Her amnestic syndrome improved and she was discharged on oral steroids. Both patients tested positive for leucine-rich glioma inactivated 1 (LGi1) antibodies. We present the first case reports of FBD episodes and drop attacks owing to LGi1 encephalitis from India and review the relevant literature pertinent to the subject.
Anti-leucine-rich glioma inactivated 1 antibodies; drop attacks; faciobrachial dystonic seizures; leucine-rich glioma inactivated 1 encephalitis; paroxysmal non-kinesogenic dystonia
Autoimmune limbic encephalitis (LE) associated with voltage gated potassium channel antibodies (VGKC-Abs) in children is more common than previously thought and is not always paraneoplastic. Non-neoplastic, autoimmune LE associated with VGKC-Abs has been described recently. However, only few case reports in children as the disease is predominantly described in the adult population. It is likely that this type of autoimmune encephalitis is currently under-diagnosed and hence, under-treated, especially in children. We present a 13-year-old previously fit and healthy African girl diagnosed with LE and we reviewed the literature for its current management.
Limbic encephalitis; paraneoplastic; voltage gated potassium channel antibodies
The objective of this study was to compare the occurrence and severity of behavioral and psychological symptoms of dementia (BPSD) between vascular dementia (VaD) and vascular cognitive impairment-no dementia (VCI-ND).
Materials and Methods:
Consecutive patients presenting with cognitive impairment at least 3 months after an ischemic stroke and with a Hachinski Ischemic Score ≥4 were included. VaD was diagnosed as per National Institute of Neurological Disorders and Stroke – Association Internationale pour la Recherche et l’Enseignement en Neurosciences criteria for probable VaD and VCI-ND on the lines of the Canadian study of health and aging. The severity of cognitive impairment and the behavioral/psychological symptoms were studied by means of the clinical dementia rating scale and the neuropsychiatric inventory (NPI) respectively.
All patients with VaD and 89% of those with VCI-ND had at least one BPSD. The mean no. of symptoms per patient and the total NPI scores were higher in VaD than in VCI-ND. Apathy and night-time behavior disturbances were significantly more common and severe in VaD.
BPSD are very common both in VCI-ND and in VaD. The profile of BPSD is similar in both groups, albeit more severe in VaD. The net burden of BPSD is higher in VaD as compared to VCI-ND.
Behavioral and psychological symptoms; neuropsychiatric inventory; vascular cognitive impairment; vascular cognitive impairment-no dementia; vascular dementia
The aim of this study was to explore the effect of adeno-associated virus (AAV) serotype 2 vector vaccine containing amyloid-β peptide (Aβ) 1-15 gene fragment (AAV-Aβ15) immunized mice sera on counteracting Aβ1-42 peptide toxicity towards a primary culture cortical neurons.
Materials and Methods:
BALB/c mice were vaccinated via the intramuscular immunization route with AAV-Aβ15. The anti-Aβ antibody titer of immunized mice sera was quantified by sandwich Enzyme-Linked ImmunoSorbent Assay. The toxicity of Aβ1-42 peptide on neurons was assessed by morphology with an inverse microscopy and cell viability was determined by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide (MTT) assay.
AAV-Aβ15 could induce an Aβ-specific immunoglobulin G (IgG) humoral immune response in /c mice the anti-Aβ antibodies were detectable at 1 month after immunization, significantly increased at 2 and 4 months after immunization, and the immunized sera could attenuate cytotoxicity of Aβ1-42 peptide on primary culture cortical neurons.
The immune serum of AAV-Aβ15 could play a neuroprotective effect against Aβ1-42 peptide toxicity, which would be beneficial for Alzheimer's disease patients.
Alzheimer's disease; amyloid-beta; immunotherapy; vaccine
The objective of this study is to assess the safety, feasibility and clinical value of transcranial motor evoked potential (MEP) monitoring by electrical stimulation.
Clinical neurophysiology department of tertiary reach hospital.
Materials and Methods:
MEP monitoring was attempted in 44 “high risk” patients. Intraoperative surgical, anesthesia and neurophysiological findings were documented prospectively. MEP monitoring results were correlated with motor outcome.
The success for reliable MEP recording from the lower limbs was 75%. Incidence of new permanent post-operative motor deficit was zero. Nearly, 76.5% of the cases (13 out of 17 cases) who showed unobtainable and unstable MEP outcome had lesion location in the spine as compared with 23.5% (4 out of 17 cases) that had lesion location in the brain. Chi-square test demonstrated a statistically significant difference between these two groups (P = 0.0020). Out of these 13 spine surgery cases, 8 (62%) were operated for deformity. Seven out of 12 (60%) patients less than 12 years of age had a poor MEP monitoring outcome suggesting that extremes of age and presence of a spine deformity may be associated with a lesser incidence of successful MEP monitoring. No complications related to the repetitive transcranial electrical stimulation for eliciting MEP were observed.
MEP monitoring is safe. The protocol used in this study is simple, feasible for use and has a fairly high success rate form the lower limbs. Pediatric age group and spine lesions, particularly deformities have an adverse effect on stable MEP recording.
Clinical and neurophysiological correlation; high-risk cases; motor evoked potential
Visual evoked potentials are useful in investigating the physiology and pathophysiology of the human visual system. Flash visual evoked potential (FVEP), though technically easier, has less clinical utility because it shows great variations in both latency and amplitude for normal subjects.
To study the effect of eye closure, low luminance, and monochromatic stimulation on the variability of FVEPs.
Subjects and Methods:
Subjects in self-reported good health in the age group of 18-30 years were divided into three groups. All participants underwent FVEP recording with eyes open and with white light at 0.6 J luminance (standard technique). Next recording was done in group 1 with closed eyes, group 2 with 1.2 and 20 J luminance, and group 3 with red and blue lights, while keeping all the other parameters constant. Two trials were given for each eye, for each technique. The same procedure was repeated at the same clock time on the following day.
Variation in FVEP latencies between the individuals (interindividual variability) and the variations within the same individual for four trials (intraindividual variability) were assessed using coefficient of variance (COV). The technique with lower COV was considered the better method.
Recording done with closed eyes, 0.6 J luminance, and monochromatic light (blue > red) showed lower interindividual and intraindividual variability in P2 and N2 as compared to standard techniques.
Low luminance flash stimulations and monochromatic light will reduce FVEP latency variability and may be clinically useful modifications of FVEP recording technique.
Eye closure; flash visual evoked potentials; latency variability; luminance; monochromatic light; visual evoked potentials
The diagnostic accuracy of the currently available tools carries poor sensitivity resulting in significant delay in specific diagnosis of cortical dementias. Considering the properties of default mode networking of the brain it is highly probable that specific changes may be seen in frontotemporal dementias (FTDs) and Alzheimer's disease sufficiently early.
The aim of this study is to look for changes in Transcranial Magnetic Stimulation (TMS) in cortical dementia.
Materials and Methods:
Evaluated with a single pulse TMS with the figure of eight coil and recorded from right first dorsal interossei (FDI). Resting Motor Threshold (RMT) was estimated on the opposite motor cortex (T1). Second site of stimulation was cervical spine at C7-T2. Central motor conduction time (CMCT) is equal toT1-T2. Silent Period (SP) identified by applying TMS pulse to contracting FDI.
RMT was reduced in seven out of eight Alzheimer's dementias. CMCT was in the upper limit of normal in both patients with FTD. The most consistent observation was that SP was reduced and there were escape discharges noticed during the SP suggesting increased cortical excitability and decreased cortical inhibition. This suggests probable early asymptomatic changes in the gamma-aminobutyric acid (GABA) nergic and cholinergic system is taking place. This if confirmed may give some insight into early diagnosis and therapeutic role of GABA agonists in these disorders.
Alzheimer's dementia; central motor conduction time; cortical inhibition; fronto-temporal dementias; transcranial magnetic stimulation
The low correlation between the patients’ signs and symptoms of carpal tunnel syndrome (CTS) and results of electrodiagnostic tests makes the diagnosis challenging in mild cases. Interpolation is a mathematical method for finding median nerve conduction velocity (NCV) exactly at carpal tunnel site. Therefore, it may be helpful in diagnosis of CTS in patients with equivocal test results.
The aim of this study is to evaluate interpolation method as a CTS diagnostic test.
Settings and Design:
Patients with two or more clinical symptoms and signs of CTS in a median nerve territory with 3.5 ms ≤ distal median sensory latency <4.6 ms from those who came to our electrodiagnostic clinics and also, age matched healthy control subjects were recruited in the study.
Materials and Methods:
Median compound motor action potential and median sensory nerve action potential latencies were measured by a MEDLEC SYNERGY VIASIS electromyography and conduction velocities were calculated by both routine method and interpolation technique.
Statistical Analysis Used:
Chi-square and Student's t-test were used for comparing group differences. Cut-off points were calculated using receiver operating characteristic curve.
A sensitivity of 88%, specificity of 67%, positive predictive value (PPV) and negative predictive value (NPV) of 70.8% and 84.7% were obtained for median motor NCV and a sensitivity of 98.3%, specificity of 91.7%, PPV and NPV of 91.9% and 98.2% were obtained for median sensory NCV with interpolation technique.
Median motor interpolation method is a good technique, but it has less sensitivity and specificity than median sensory interpolation method.
Carpal tunnel syndrome; electrodiagnosis; interpolation; nerve conduction velocity
Poor documentation following lumbar puncture (LP) had always been a matter of concern. This study aimed to investigate the documentation pattern of neurology house officers, registrars (Regs), and specialist Regs following LP in a major teaching hospital.
Materials and Methods:
Total hundred patient records were examined in the light of a carefully designed proforma containing 15 important indicators of good-quality LP documentation.
Mean number of indicators overall documented by doctors was found to be 6.24 ± 3.0037. The mean number of indicators recorded by house officers was 5.11 ± 3.01 and Regs was 7.56 ± 3.28. A total of 33% LPs were performed without a documented consent. Only 36% performers documented the type and size of needle they used during the procedure. Only 46% documents revealed the strength and name of the local anesthetic used. Statistically significant difference between senior house officers and Regs in terms of numbers of indicators documented was noted.
The documentation standard among neurology junior doctors remained poor.
Consent; documentation; knowledge; lumbar; neurology; puncture
Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE.
We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken's criteria. We analyzed demographical, clinical, electrophysiological, and imaging features.
Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging.
SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.
Measles; myoclonus; subacute sclerosing panencephalitis
To evaluate the effectiveness of mirror therapy on lower extremity motor recovery, balance and mobility in patients with acute stroke.
A randomized, sham-controlled, assessor blinded, pilot trial.
Inpatient stroke rehabilitation unit.
First time onset of stroke with mean post-stroke duration of 6.41 days, able to respond to verbal instructions, and Brunnstrom recovery stage 2 and above were enrolled.
Mirror therapy group performed 30 minutes of functional synergy movements of non-paretic lower extremity, whereas control group underwent sham therapy with similar duration. In addition, both groups were administered with conventional stroke rehabilitation regime. Altogether 90 minutes therapy session per day, six days a week, for two weeks duration was administered to both groups.
Lower extremity motor subscale of Fugl Meyer Assessment (FMA), Brunnel Balance Assessment (BBA) and Functional Ambulation Categories (FAC).
Amongst the 22 patients included, equal number of patients participated in mirror group (N = 11) and control group (N = 11). Baseline variables were similar in both groups, except for Brunnstrom recovery stage. There was no statistical difference between groups, except for FAC. (FMA: P = 0.894; BBA: P = 0.358; FAC: P = 0.02). Significance was set at P < 0.05.
Administration of mirror therapy early after stroke is not superior to conventional treatment in improving lower limb motor recovery and balance, except for improvement in mobility.
Acute stroke; balance; mobility; mirror therapy; motor recovery
Studies have suggested that dementia is differentially distributed across populations with a lower prevalence in developing regions than the developed ones. A comparison in the prevalence of dementia across populations may provide an insight into its risk factors. Keeping this in view, a study was planned to evaluate the prevalence of dementia in tribal elderly population.
Materials and Methods:
A cross-sectional comprehensive two-phase survey of all residents aged 60 years and older was conducted. Phase one involved screening of all individuals aged 60 and above with the help of a cognitive screen specifically developed for the tribal population. Phase two involved clinical examination of individuals who were suspected of dementia as per the developed cognitive screening test.
The results revealed that no individual above 60 years of age in the studied population was diagnosed as a case of dementia. Thereby, pointing out at some unknown factors, which are responsible for prevention of dementia.
The differences between the prevalence rate in this study and other studies in India appear to be a function of a valid regional difference. Environmental, phenotypic and genetic factors may contribute to regional and racial variations in dementia. Societies living in isolated hilly and tribal areas seem less predisposed to dementia, particularly age related neurodegenerative and vascular dementia, which are the most common causes for dementia in elderly. This may be because some environmental risk factors are much less prevalent in these settings.
Dementia; populations; risk; tribal
To know the incidence of bacterial meningitis in children below five years of age. To compare conventional culture and antigen detection methods (Latex agglutination test).
Materials and Methods:
100 CSF samples of clinically suspected meningitis cases in children below 5 years of age were included. The samples were subjected to cell count, Gram stain, culture and LAT. The organisms isolated in the study were characterized according to standard procedures.
Of the 100 cases studied, 31 cases were diagnosed as ABM by Gram stain, culture and latex agglutination test as per WHO criteria. The hospital frequency of ABM was 1.7%. 15 (48.38) cases were culture positive. Gram stain was positive in 22(70.96) cases and LAT in 17(54.83) cases. Haemophilus influenzae was the most common causative agent of acute bacterial meningitis followed by S.pneumoniae. Case fatality rate was 45.16%. The sensitivity and specificity of LAT was 66.66% and 87.91% respectively.
Bacterial meningitis is a medical emergency and early diagnosis and treatment is life saving and reduces chronic morbidity. LAT was more sensitive compared to conventional Gram stain and Culture technique in identifying the fastidious organisms like H.influenzae, S.pneumoniae and Group B Streptococcus. However, the combination of Gram stain, Culture and LAT proved to be more productive than any of the single tests alone.
Acute bacterial meningitis; culture; latex agglutination
Headache, musculoskeletal symptoms, and vitamin D deficiency are common in the general population. However, the interrelations between these three have not been delineated in the literature.
Materials and Methods:
We retrospectively studied a consecutive series of patients who were diagnosed as having chronic tension-type headache (CTTH) and were subjected to the estimation of serum vitamin D levels. The subjects were divided into two groups according to serum 25(OH) D levels as normal (>20 ng/ml) or vitamin D deficient (<20 ng/ml).
We identified 71 such patients. Fifty-two patients (73%) had low serum 25(OH) D (<20 ng/dl). Eighty-three percent patients reported musculoskeletal pain. Fifty-two percent patients fulfilled the American College of Rheumatology criteria for chronic widespread pain. About 50% patients fulfilled the criteria for biochemical osteomalacia. Low serum 25(OH) D level (<20 ng/dl) was significantly associated with headache, musculoskeletal pain, and osteomalacia.
These suggest that both chronic musculoskeletal pain and chronic headache may be related to vitamin D deficiency. Musculoskeletal pain associated with vitamin D deficiency is usually explained by osteomalacia of bones. Therefore, we speculate a possibility of osteomalacia of the skull for the generation of headache (osteomalacic cephalalgia?). It further suggests that both musculoskeletal pain and headaches may be the part of the same disease spectrum in a subset of patients with vitamin D deficiency (or osteomalacia), and vitamin D deficiency may be an important cause of secondary CTTH.
Chronic daily headache; chronic musculoskeletal pain; chronic pain; chronic tension-type headache; osteomalacia; pain; vitamin D
Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diagnostic criterion for sporadic CJD (sCJD) is useful for making an early diagnosis.
To report a series of patients of probable sCJD from a neurology institute of eastern India.
Materials and Methods:
Patients of rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause including electroencephalography (EEG), magnetic resonance imaging (MRI) of brain, and cerebrospinal fluid analysis.
A total 10 patients of probable sCJD diagnosed using the European diagnostic criterion between December 2011 and January 2013. The clinical features are consistent with other reported series. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Eight patients died within a mean duration of 4.56 months from the disease onset.
The clinical features are similar to other reported series. Our observation raises question about the prevalence of this disease in India which needs more elaborate studies.
Creutzfeldt-Jakob disease; electroencephalography; magnetic resonance imaging
Rhabdomyolysis results from many causes including hypernatremia. Postpartum hypernatremia with osmotic cerebral demyelination is a rare cause of reversible rhabdomyolysis. Electromyographic studies in postpartum hypernatremia have not been reported.
Materials and Methods:
Electromyography (EMG) was performed in five women with postpartum hypernatremia and muscle biopsy was performed in one of them.
Among the five women presenting with postpartum hypernatremia associated with marked elevation of serum creatine kinase, four had quadriparesis. All had varying degrees of encephalopathy at admission and recovered without residual deficits after gradual correction of hypernatremia. Needle EMG revealed fibrillations with positive sharp waves in five patients and myotonic discharges in three patients. Serial EMG in one patient revealed the occurrence of transient fibrillations, positive sharp waves and myotonic discharges. Muscle biopsy revealed extensive rhabdomyolysis in one patient.
EMG in hypernatremic rhabdomyolysis revealed spontaneous activity including fibrillations, positive sharp waves and myotonic discharges along with myopathic potentials. Electromyographic findings depend on the interval from the onset and the degree of rhabdomyolysis.
Electromyography; hypernatremia; myotonic discharges; rhabdomyolysis; postpartum
Ictal Pet in presurgical workup of refractory epilepsy is seldom performed and limited due to technical difficulties. In carefully selected patient subset with frequent extratemporal seizures, ictal PET depicts ‘seizure onset zone’ with high spatial resolution even within a widespread pathology. We here depict a four year old with posterior quadrant dysplasia evaluated with ictal PET.
Epilepsy; Ictal PET; presurgical workup; refractory
Polymicrogyria is a type of cortical dysplasia with cortical organizational defect. Bilateral polymicrogyria are distinct with genetic basis in a subset. We hereby report a case of bilateral frontal polymicrogyria (BFP) in association with chorioretinal dystrophy and ectopia lentis (EL) in a 26-year-old lady born of a consanguineous parentage. Her male sibling also had chorioretinal dystrophy and EL. This combination of autosomal recessive inheritance has not been reported earlier in the literature and suggests a role of connective tissue genes in BFP.
Autosomal recessive; chorioretinal-dystrophy; ectopia lentis; frontal-polymicrogyria
Neurological melioidosis is a very rare and very few cases have been reported from India. Presentation is an extremely varied and as this disease is associated with high mortality, high index of suspicion is needed to diagnose and treat. In this context, we report a patient presenting as Guillain Barre syndrome evaluated as melioidosis.
Guillain Barre’ syndrome; infection; melioidosis
Peduncular hallucinosis usually occurs due to vascular or infectious midbrain lesions or brain stem compression by tumors. We present a peduncular hallucinosis case in a 63-year-old female with brain stem infarction, which can easily be misdiagnosed as a psychiatric disorder.
Cerebrovascular accident; peduncular hallucinosis; midbrain