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1.  Comparative study of thyrotoxic periodic paralysis from idiopathic hypokalemic periodic paralysis: An experience from India 
Objective:
There is paucity of reports on thyrotoxic periodic paralysis (TPP) from India. We report the patients with TPP and compare them with idiopathic hypokalemic periodic paralysis (IHPP).
Materials and Methods:
Patients with hypokalemic periodic paralysis (HPP) treated during the past 11 years were evaluated retrospectively. Their demographic parameters, family history, clinical features, precipitating factors, severity of weakness, laboratory parameters and rapidity of recovery were recorded. The demographic, clinical and laboratory parameters of TPP and IHPP were compared.
Results:
During the study period, we managed 52 patients with HPP; nine (17.3%) of whom had TPP and 27 (52%) had IHPP. The demographic, precipitating factors, number of attacks and severity of limb weakness were similar between the TPP and IHPP groups, except in the IHPP group, bulbar weakness was present in four and respiratory paralysis in six, needing artificial ventilation in two patients. Serum potassium was significantly lower in TPP (2.21 ± 0.49) compared with IHPP (2.67 ± 0.59, P = 0.04). Four patients with TPP had subclinical thyrotoxicosis and two had subclinical hyperthyroidism. Rebound hyperkalemia occurred in both TPP and IHPP (three versus eight patients). The recovery was faster in IHPP (26.7 ± 15.4 h) compared with TPP (34.0 ± 14.0 h), but was statistically insignificant.
Conclusion:
TPP constitutes 17.3% of HPP, and absence of clinical features of thyrotoxicosis and subclinical hyperthyroidism in TPP is not uncommon. Clinical features, demographic profile and rebound hyperkalemia are similar in both TPP and IHPP. The serum potassium level is significantly low in the TPP compared with the IHPP group.
doi:10.4103/0972-2327.99708
PMCID: PMC3424795  PMID: 22919190
Hypokalemia; hypokalemic periodic paralysis; periodic paralysis; thyrotoxic periodic paralysis; treatment
2.  Comparison of clinical and electrodiagnostic features in B12 deficiency neurological syndromes with and without antiparietal cell antibodies 
Postgraduate Medical Journal  2007;83(976):124-127.
Background and aims
This study was undertaken to compare the clinical and electrodiagnostic (Edx) features in autoimmune and nutritional vitamin B12 deficiency neurological syndromes.
Methods
Consecutive patients with vitamin B12 deficiency neurological syndromes were evaluated and blood counts, red blood cell indices, serum chemistry, thyroid function, HIV serology, antiparietal cell antibody (APCA), serum B12, bone marrow and spinal MRI assessed. EDx studies included nerve conduction, tibial somatosensory (SEP) and motor evoked potential (MEP) to the tibialis anterior, and visual evoked potential (VEP). The results were compared between APCA positive and negative groups.
Results
57 patients aged 17–80 years (mean 45.3) were studied; 48 were vegetarians. The presenting clinical syndromes were myeloneuropathy in 25, myelopathy in 14, myeloneuroencephalopathy in 13, myeloencephalopathy in four and behavioural abnormality only in one patient. Spinal MRI in 47 patients revealed posterior spinal cord hyperintensity in 21 and cord atrophy in six. Nerve conduction was abnormal in 15%, MEP in 56.6%, SEP in 87.3% and VEP in 63.6% of patients. At 3 months, 31 patients had complete, 11 partial and three poor recovery. APCA was positive in 49% of patients. There was no difference in clinical, MRI or Edx findings or outcome between the APCA positive and negative groups.
Conclusion
APCA was positive in 49% of patients with B12 deficiency neurological syndrome but their clinical, MRI and Edx changes were not different from the APCA negative group. Neurological manifestations may be caused by B12 deficiency itself rather than the underlying cause.
doi:10.1136/pgmj.2006.048132
PMCID: PMC2805935  PMID: 17308217
3.  Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease 
Background
Flexion myelopathy is one of the suggested mechanism for Hirayama disease (HD) but simultaneous radiological and neurophysiological evaluation is lacking. This study therefore evaluates the effect of neck flexion in HD using somatosensory evoked potentials (SEPs), F waves, and magnetic resonance imaging (MRI).
Method
Eight HD patients and seven matched controls were subjected to median and ulnar F wave (minimal latency, FM ratio, persistence, and chronodispersion), and SEPs evaluating N9, N13, and N20 potentials in neutral and neck flexion. Spinal MRI was carried out in neutral and neck flexion and evaluated for cord atrophy, signal changes, cord compression, posterior epidural tissue, and loss of dural attachment.
Results
The patients were aged 19 to 30 years. Minimal F latency, FM ratio, persistence, and chronodispersion in neutral and neck flexion did not show any change nor was there any change in N13 latency and amplitude on median and ulnar SEPs. The difference in these parameters in neutral and neck flexion were also not significant in HD compared with controls. The change in N13 was also not related to loss of dural attachment and posterior epidural tissue.
Conclusion
Neck flexion does not produce significant changes in N13 and F wave parameters and is not related to dynamic MRI changes. The other mechanisms for HD should therefore be explored.
doi:10.1136/jnnp.2005.082362
PMCID: PMC2117439  PMID: 16614039
F wave; Hirayama disease; magnetic resonance imaging; somatosensory evoked potential; neck flexion
4.  Diabetic neuropathy 
Postgraduate Medical Journal  2006;82(964):95-100.
Diabetic neuropathy (DN) refers to symptoms and signs of neuropathy in a patient with diabetes in whom other causes of neuropathy have been excluded. Distal symmetrical neuropathy is the commonest accounting for 75% DN. Asymmetrical neuropathies may involve cranial nerves, thoracic or limb nerves; are of acute onset resulting from ischaemic infarction of vasa nervosa. Asymmetric neuropathies in diabetic patients should be investigated for entrapment neuropathy. Diabetic amyotrophy, initially considered to result from metabolic changes, and later ischaemia, is now attributed to immunological changes. For diagnosis of DN, symptoms, signs, quantitative sensory testing, nerve conduction study, and autonomic testing are used; and two of these five are recommended for clinical diagnosis. Management of DN includes control of hyperglycaemia, other cardiovascular risk factors; α lipoic acid and L carnitine. For neuropathic pain, analgesics, non‐steroidal anti‐inflammatory drugs, antidepressants, and anticonvulsants are recommended. The treatment of autonomic neuropathy is symptomatic.
doi:10.1136/pgmj.2005.036137
PMCID: PMC2596705  PMID: 16461471
neuropathy; diabetes; treatment; classification; pathophysiology
5.  A study of computed tomography scan and magnetic resonance imaging findings in pseudohypoparathyroidism 
We report three children with pseudohypoparathyroidism aged 13-16 years who presented with seizures and tetany. CT scan revealed striatopallidal calcification in two. MRI revealed wide-spread involvement showing T1 hyperintensity in striatopallidodentate distribution in all three and midbrain in one patient. T2 and FLAIR images were normal. T1 hyperintensity could represent early stage of calcification in whom MRI is more sensitive.
doi:10.4103/0972-2327.70870
PMCID: PMC2981760  PMID: 21085533
CT scan; MRI; parathormone; pseudohypoparathyroidism
6.  Loss of cell surface TFII-I promotes apoptosis in prostate cancer cells stimulated with activated α2-macroglobulin* 
Journal of cellular biochemistry  2011;112(6):1685-1695.
Receptor-recognized forms of α2-macroglobulin (α2M*) bind to cell surface-associated GRP78 and initiate pro-proliferative and anti-apoptotic signaling. Ligation of GRP78 with α2M* also upregulates TFII-I, which binds to the GRP78 promoter and enhances GRP78 synthesis. In addition to its transcriptional functions, cytosolic TFII-I regulates agonist-induced Ca2+ entry. In this study we show that down regulation of TFII-I gene expression by RNAi profoundly impairs its cell surface expression and anti-apoptotic signaling as measured by significant reduction of GRP78, Bcl-2, and cyclin D1 in 1-Ln and DU-145 human prostate cancer cells stimulated with α2M*. In contrast, this treatment significantly increases levels of the pro-apoptotic proteins p53, p27, Bax, and Bak and causes DNA fragmentation. Furthermore, down regulation of TFII-I expression activates agonist-induced Ca2+ entry. In plasma membrane lysates p-PLCγ1, TRPC3, GRP78, MTJ1, and caveolin co-immunoprecipitate with TFII-I suggesting multimeric complexes of these proteins. Consistent with this hypothesis, down regulating TFII-I, MTJ1, or GRP78 expression by RNAi greatly attenuates cell surface expression of TFII-I. In conclusion, we demonstrate that not only does cell surface GRP78 regulate apoptosis, but it also regulates Ca2+ homeostasis by controlling cell surface localization of TFII-I.
doi:10.1002/jcb.23083
PMCID: PMC3080239  PMID: 21503958
Prostate cancer cells; activated α2-macroglobulin*; TFII-I; TRPC signaling complex; Ca2+ influx; apoptosis
7.  Radiological and neurophysiological changes in Japanese encephalitis. 
Six patients with Japanese encephalitis, four males and two females whose age ranged between 2 and 47 years, were subjected to neurophysiological and radiological studies. An EEG in five of the patients showed diffuse delta wave activity and one had an alpha coma. Delta activity seems to be due to thalamic involvement, which was seen on CT of two and MRI of all the patients. The thalamic lesions were characteristically bilateral and were haemorragic in five. Changes on MRI included abnormalities of the brainstem in three and the basal ganglia and spinal cord in one patient each. Lower motor neuron signs were present in three patients but abnormal MRI signals in the spinal cord were present in only one out of three patients in whom spinal MRI was carried out. Central motor conduction time in the upper limb was prolonged in three patients (five sides) and in the lower limbs in one (both sides), which was consistent with involvement of the cerebral cortex, thalamus, brainstem, and spinal cord. Changes in MRI and EEG in the acute stage may provide early diagnostic clues in patients with Japanese encephalitis.
Images
PMCID: PMC1073229  PMID: 7798977
8.  Peripheral and central conduction studies in neurolathyrism. 
To study the involvement of motor and sensory pathways in neurolathyrism, 19 patients with lathyrism from Unnao, India, where lathyrism is endemic, were studied. The mean age of the patients at the time of the onset of illness was 35.8 (range 18-70) years. The mean duration of illness was 15.6 (range 2-30) years. The clinical picture comprised walking difficulty due to stiffness and mild weakness in all 19 patients, cramps in the legs in five, frequency or urgency of micturition in five, and flexor spasms in three. There was pronounced leg spasticity with a mean Ashworth score of 4.1 (range 2.9-5). Central motor conduction to the tibialis anterior muscle (CMCT-TA) was slow in 14 of the 17 patients (21 sides). Slowing of peripheral motor nerve conduction, although less pronounced, was significant in the upper limb in four and the lower limb in seven sides. The tibial somatosensory evoked potentials were normal and peroneal nerve conduction was marginally impaired. Values for CMCT-TA correlated with the degree of spasticity (p < 0.02) whereas weakness, crossed adductor reflexes, and clonus did not. The wide variability of CMCT-TA in lathyrism may be due to involvement of different types of fibres. Large diameter fibre involvement may cause pronounced slowing. Small diameter fibre involvement could produce appreciable spasticity and mild weakness but a lesser degree of slowing or even normal conduction.
Images
PMCID: PMC1072917  PMID: 8201326
9.  Magnetic resonance imaging may simulate progressive multifocal leucoencephalopathy in a patient with chronic lymphocytic leukemia after fludarabine therapy 
A 60-year-old male with chronic lymphatic leukemia (CLL) after 6 months of fludarabine therapy was admitted with status epilepticus and developed left hemiplegia. His magnetic resonance imaging revealed multiple T2 hyperintense lesions in the right frontal and left parieto-occipital lesion, simulating progressive multifocal leucoencephalopathy (PML). Cerebrospinal fluid Polymerase Chain Reaction (PCR) for JC virus was negative. We suggest the possible role of fludarabine in producing PML-like lesions in patients with Chronic Lymphocytic Leukemia (CLL).
doi:10.4103/0972-2327.41880
PMCID: PMC2771959  PMID: 19893650
Progressive multifocal leucoencephalopathy; chronic lymphocytic leukemia; fludarabine
10.  Factors predicting surgical outcome of thymectomy in myasthenia gravis: A 16-year experience 
Aim:
To assess the surgical outcome of myasthenia gravis (MG) following thymectomy and to determine the outcome predictors to such therapeutic approach.
Materials and Methods:
This study is a retrospective review of 80 consecutive thymectomies performed for MG over a 16-year period.
Results:
There were 41 females and 39 males (mean age, 34.32 years) with mean disease duration of 17.45 months prior to surgery. Stagewise distribution of the patients revealed 2.5% in stage I, 48.7% in stage IIA, 33.8% in stage IIB, 8.7% in stage III, and 6.3% in stage IV. The surgical approach was either trans-sternal (n=67) or video-assisted thoracoscopic route (n=13). Follow-up was obtained in 91.2% (n=73) of patients with mean duration of 67.7 months. At their last follow-up, 26.0% were in complete remission, 35.6% were asymptomatic on decreased medications, and 17.8% had clinical improvement on decreased medications. Overall, 79.4% of patients benefited from surgery, 8.2% had unchanged disease status, and 12.3% worsened clinically. Factors influencing favorable outcome include sex, disease stage, gland weight, and preoperative medication with anti-cholinesterase (P<0.05). There was one death in the perioperative period due to septicemia. Two patients died at fourth and seventh month following thymectomy.
Conclusion:
Thymectomy for MG is safe and effective. Certain influencing factors may shape treatment decisions and target higher risk patients.
doi:10.4103/0972-2327.91945
PMCID: PMC3271465  PMID: 22346015
Myasthenia gravis; thymectomy; video-assisted thymectomy
11.  Cerebral malaria and bacterial meningitis 
Annals of Indian Academy of Neurology  2011;14(Suppl1):S35-S39.
doi:10.4103/0972-2327.83101
PMCID: PMC3152171  PMID: 21847328
12.  Pharmacogenomic association study on the role of drug metabolizing, drug transporters and drug target gene polymorphisms in drug-resistant epilepsy in a north Indian population 
Indian Journal of Human Genetics  2011;17(Suppl 1):S32-S40.
BACKGROUND:
In epilepsy, in spite of the best possible medications and treatment protocols, approximately one-third of the patients do not respond adequately to anti-epileptic drugs. Such interindividual variations in drug response are believed to result from genetic variations in candidate genes belonging to multiple pathways.
MATERIALS AND METHODS:
In the present pharmacogenetic analysis, a total of 402 epilepsy patients were enrolled. Of them, 128 were diagnosed as multiple drug-resistant epilepsy and 274 patients were diagnosed as having drug-responsive epilepsy. We selected a total of 10 candidate gene polymorphisms belonging to three major classes, namely drug transporters, drug metabolizers and drug targets. These genetic polymorphism included CYP2C9 c.430C>T (*2 variant), CYP2C9 c.1075 A>C (*3 variant), ABCB1 c.3435C>T, ABCB1c.1236C>T, ABCB1c.2677G>T/A, SCN1A c.3184 A> G, SCN2A c.56G>A (p.R19K), GABRA1c.IVS11 + 15 A>G and GABRG2 c.588C>T. Genotyping was performed using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) methods, and each genotype was confirmed via direct DNA sequencing. The relationship between various genetic polymorphisms and responsiveness was examined using binary logistic regression by SPSS statistical analysis software.
RESULTS:
CYP2C9 c.1075 A>C polymorphism showed a marginal significant difference between drug resistance and drug-responsive patients for the AC genotype (Odds ratio [OR] = 0.57, 95% confidence interval [CI] = 0.32–1.00; P = 0.05). In drug transporter, ABCB1c.2677G>T/A polymorphism, allele A was associated with drug-resistant phenotype in epilepsy patients (P = 0.03, OR = 0.31, 95% CI = 0.10-0.93). Similarly, the variant allele frequency of SCN2A c.56 G>A single nucleotide polymorphism was significantly higher in drug-resistant patients (P = 0.03; OR = 1.62, 95% CI = 1.03, 2.56). We also observed a significant difference at the genotype as well as allele frequencies of GABRA1c.IVS11 + 15 A > G polymorphism in drug-resistant patients for homozygous GG genotype (P = 0.03, OR = 1.84, 95% CI = 1.05–3.23) and G allele (P = 0.02, OR = 1.43, 95% CI = 1.05–1.95).
CONCLUSIONS:
Our results showed that pharmacogenetic variants have important roles in epilepsy at different levels. It may be noted that multi-factorial diseases like epilepsy are also regulated by various other factors that may also be considered in the future.
doi:10.4103/0971-6866.80357
PMCID: PMC3125053  PMID: 21747585
Drug resistance; epilepsy; pharmacogenomics
13.  Altered interaction of Cis-dichlorodiammineplatinum(II)--modified alpha 2-macroglobulin (alpha 2M) with the low density lipoprotein receptor-related protein/alpha 2M receptor but not the alpha 2M signaling receptor. 
Journal of Clinical Investigation  1996;97(5):1193-1203.
Receptor-recognized forms of alpha 2-macroglobulin (alpha 2M*) bind to two macrophage receptors: an endocytic receptor, the low density lipoprotein receptor-related protein/alpha 2M receptor (LRP/alpha 2MR), and a G protein-coupled receptor, the alpha 2M signaling receptor (alpha 2MSR). Binding of alpha 2M* to LRP/alpha 2MR but not alpha 2MSR is inhibited by receptor-associated protein. We now present binding characteristics of alpha 2MSR (kD approximately 50 pm; 1,530 sites/cell) using Scatchard analysis. We also demonstrate that chemical modification of alpha 2M* with cis-dichlorodiammineplatinum (cis-DDP) does not significantly alter binding to either receptor or signaling characteristics as compared with unmodified alpha 2M*. However, internalization by LRP/alpha 2MR is greatly affected. Cis-DDP-modified alpha 2M* (cis-DDP-alpha 2M*) and alpha 2M* show comparable internalization during a single round of endocytosis; however, cis-DDP modification of alpha 2M* results in a > or = 82% reduction in internalization involving receptor recycling and multiple rounds of endocytosis. Results from pH 5.0 dissociation and receptor recycling experiments suggest that the mechanism of decreased internalization of cis-DDP-alpha 2M* involves poor dissociation from the receptor in endosomes and a decrease in available surface receptors over the time of exposure to the ligand.
PMCID: PMC507171  PMID: 8636430
14.  Postural hypotension in a patient with acute myelitis. 
Postgraduate Medical Journal  1996;72(845):180-182.
A case of acute transverse myelitis with severe postural hypotension is described.
Images
PMCID: PMC2398402  PMID: 8731714

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