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2.  Hot-water epilepsy: From bench to bedside 
doi:10.4103/0972-2327.112442
PMCID: PMC3724060  PMID: 23956550
3.  Idiopathic generalized epilepsy: Phenotypic and electroencephalographic observations in a large cohort from South India 
Purpose:
We studied the phenotype and electroencephalographic (EEG) features, and therapeutic aspects of idiopathic generalized epilepsies (IGEs) in South Indian population.
Patients and Methods:
This prospective cross-sectional hospital-based study was carried out on non-consecutive 287 patients (age 22.2 ± 7.7 years; M:F = 139:148) with IGE syndrome. Their clinical and EEG observations were analyzed.
Results:
Majority of the patients had onset of seizures <20 years of age (n = 178; 62%). Thirty one patients (10.8%) had family history of epilepsy. Nearly half of them (49.9%) had <5 years of duration of seizures. The type of IGEs included Juvenile myoclonic epilepsy (JME): 115 (40.1%); IGE with generalized tonic-clonic seizures (GTCS) only: 102 (39.02%); childhood absence epilepsy (CAE): 35 (12.2%); GTCS on awakening: 15 (5.2%); Juvenile absence epilepsy (JAE): 11 (3.8%); and unclassified seizures: 9 (3.1%). The triggering factors noted in 45% were sleep deprivation (20%), non-compliance and stress in 5% each. The EEG (n = 280) showed epileptiform discharges in about 50% of patients. Epileptiform discharges during activation was observed in 40/249 patients (16.1%): Hyperventilation in 32 (12.8%) and photic stimulation in 19 (7.6%). The seizures were well controlled with anti-epileptic drugs (AEDs) in 232 (80.8%) patients and among them, 225 (78.4%) patients were on monotherapy. Valproate (n = 131) was the most frequently prescribed as monotherapy.
Conclusions:
This is one of the largest cohort of patients with IGE. This study reiterates the importance of segregating IGE syndrome and such analysis will aid to the current understanding and management.
doi:10.4103/0972-2327.112455
PMCID: PMC3724066  PMID: 23956556
Electroencephalographic; idiopathic generalized epilepsy; epilepsy syndromes; seizure types
4.  Neuroimaging observations in a cohort of elderly manifesting with new onset seizures: Experience from a university hospital 
Background:
The occurrence of epilepsy is higher among elderly patients. The clinical manifestations of seizures, causes of epilepsy, and choice of anti-epileptic drugs (AEDs) are different in elderly people with epilepsy compared to the young.
Aim:
To evaluate the imaging (CT/MRI) observations in elderly patients manifesting with new-onset seizures.
Materials and Methods:
Two hundred and one elderly patients with new onset seizures, >60 years (age: 68.0 ± 7.5 years; M:F = 1.8:1) from Jan’ 07 to Jan’ 09, were prospectively recruited. Observations of cranial CT scan (n = 201) and MR imaging (n = 43) were analyzed.
Results:
The type of seizures included: Simple partial (42%), generalized tonic-clonic (30.3%), and complex partial (27.4%). The pattern of epilepsy syndromes were acute symptomatic (42.3%), remote symptomatic (18.4%), cryptogenic (37.8%), and idiopathic (1.5%). Seizures were controlled with monotherapy in 85%. The CT scan (n = 201) revealed cerebral atrophy (139), mild (79), moderate (43), and severe (18); focal lesions (98), infarcts (45), hemorrhages (18), granuloma (16), tumor (15) and gliosis (4), and hemispheric atrophy (1), white matter changes (75) and diffuse edema (21). An MRI (n = 43) showed variable degree of cerebral atrophy (31); white matter changes (20); focal cerebral lesions (24); - infarct (7); intracranial hemorrhage (6); granuloma (5); tumor (6); gliosis (1); hemispheric atrophy (1); and prominent Virchow-Robin spaces (7); and UBOs (12). Patients with focal lesions in neuroimaging more often had partial seizures, symptomatic epilepsy, past stroke, focal deficit, absence of diffuse atrophy, focal EEG slowing, abnormal CSF, seizure recurrence at follow-up (P < 0.05).
Conclusions:
Brain imaging observations in elderly patients with new-onset seizures revealed underlying symptomatic nature, hence the etiology and thereby assisted in deciding the specific therapy.
doi:10.4103/0972-2327.104335
PMCID: PMC3548365  PMID: 23349592
Computerized tomographyt; Magnetic resonance imaging; seizures in elderly
5.  Steroid-responsive encephalopathy in autoimmune thyroiditis: Clinical spectrum and MRI observations in three cases 
Hashimoto's encephalopathy (H.E.) is probably of autoimmune etiology, and manifests with seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, myoclonus. It is presumed to be autoimmune in origin with high serum titers of antithyroid peroxidase antibodies (anti-TPA). Thyroid function might often be normal. The diagnosis is arrived at by excluding other toxic, metabolic and infectious causes of encephalopathies, supportive clinical profile, elevated thyroid antibodies and optimum steroid response. We present the characteristic phenotypic manifestations, magnetic resonance imaging and electroechography observations and response to immunomodulation with follow-up in three cases of H.E. All the three cases manifested with subacute to chronic progressive encephalopathy, cerebellar dysfunction, seizures, behavioral abnormalities and oculomotor disturbances and had evidence of hypothyroidism, elevated titers of anti-TPA and positive thyroid anti-microsomal antibodies. Atypical and uncommon presentations are known. This report emphasizes that a high index of suspicion is often required in cases with “investigation negative encephalopathy” for early diagnosis of H.E.
doi:10.4103/0972-2327.85897
PMCID: PMC3200047  PMID: 22028537
Antithyroid peroxidase antibodies; Hashimoto's encephalopathy
6.  Sleep in Wilson’s disease: Questionnaire based study 
Objective:
We proposed to detect sleep abnormalities in Wilson’s disease, (WD) using sleep questionnaires.
Materials and Methods:
Twenty-five patients (M:F = 18:7; age: 24.4 ± 9.2 years) with WD and 24 controls (all males; age: 33.1 ± 9.7 years) were recruited. They underwent phenotypic/magnetic resonance imaging (MRI) evaluation followed by administration of Pittsburg Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS) questionnaires.
Results:
The mean age at presentation and diagnosis was 24.4 ± 9.2 and 17.6 ± 7.5 years, respectively. The duration of illness at diagnosis was 14 ± 21.9 months. On PSQI, 15 patients with WD had abnormal PSQI scores of >5 as compared to 6 patients among the controls. The mean PSQI score was significantly more (P = 0.03) in patients compared to the controls. The PSQI worst scores were noted only in WD. Evaluation with ESS showed that three patients with WD scored >10/24, while two among the controls qualified for excessive daytime sleepiness. Overall, assessment by sleep questionnaires detected abnormality in 16 patients with WD as compared to 8 controls (P = 0.004). Subgroup analysis revealed that patients whose duration of illness was >8 years and who were on decoppering treatment had significantly lesser excessive daytime somnolence.
Conclusions:
Sleep disturbances were observed more often in WD than in controls. Better designed studies will provide a better understanding.
doi:10.4103/0972-2327.78047
PMCID: PMC3098521  PMID: 21633612
Epworth sleepiness scale; Pittsburg sleep quality index; sleep; sleep questionnaire; Wilson’s disease
7.  Familial amyloidotic polyneuropathy with muscle, vitreous, leptomeningeal, and cardiac involvement: Phenotypic, pathological, and MRI description 
Familial amyloidotic polyneuropathy (FAN type 1) is a rare systemic disease that causes severe and disabling peripheral neuropathy. We describe the phenotypic, radiological, and pathological characteristics of a patient with familial amyloid polyneuropathy type 1 who had evidence of motor-sensory-autonomic neuropathy, ocular vitreous deposits, diffuse leptomeningeal involvement, and hypertrophic cardiomyopathy. Muscle involvement, an infrequently reported feature, was also observed. Early recognition of the disease has significant therapeutic implications.
doi:10.4103/0972-2327.64642
PMCID: PMC2924515  PMID: 20814501
Amyloidosis; cardiomyopathy; familial amyloidotic polyneuropathy; leptomeningitis; myopathy; neuropathy; vitreous deposits
8.  Radial neuropathy due to occupational lead exposure: Phenotypic and electrophysiological characteristics of five patients 
Lead is a ubiquitous and versatile metal that has been used by mankind for many years. It is a toxic heavy metal that ranks as one of the most important environmental poisons in the world. Research conducted in recent years has increased public health concern about the toxicity of lead at low doses and has supported a reappraisal of the levels of lead exposure that may be safely tolerated in the workplace. Neuropathy is one complication of lead poisoning. The aim of this study is to describe the phenotypic and electrophysiological profile in five male patients working in a battery factory who developed radial nerve neuropathy due to lead exposure. All patients had elevated blood lead levels that were in the toxic range. The concerned regulatory bodies should make it mandatory for workers to undergo regular health checkups to detect signs of lead poisoning and must ensure that workers are aware about the ill effects of exposure to this metal. Chelation therapy removes lead from the blood and soft tissues and chronic lead exposure often requires repeated courses of treatment.
doi:10.4103/0972-2327.53080
PMCID: PMC2812735  PMID: 20142857
Electrophysiology; lead; neuropathy; radial nerve
9.  Epilepsia partialis continua in mitochondrial dysfunction: Interesting phenotypic and MRI observations 
An 11-year-old girl manifested with photophobia, ptosis, external ophthalmoplegia, hypotonia, weakness of proximal limb muscles, hyporeflexia, and generalized seizures (six months). Her elder sister had had uncontrolled seizures and photophobia and died at seven years of age. In the patient, serum lactate was high (55 mg/dl). Muscle biopsy revealed characteristic ragged red and ragged blue fibers, diagnostic of mitochondrial cytopathy. Sequencing of the complete mitochondrial genome of the DNA obtained from the muscle biopsy of the patient did not show any characteristic mutation. Four months later, the girl was admitted with a one-week history of epilepsia partialis continua (EPC). EEG revealed Periodic Lateralized Epileptiform Discharges (PLEDs), once in 2-4 seconds, over the right temporo-occipital leads. MRI revealed signal change of right motor cortex, which had restricted diffusion. MR spectroscopy (MRS) from this region revealed lactate peak. EPC remained refractory to multiple anti-epileptic drugs, immuno-modulators, coenzyme-Q, and carnitine. This thought provoking report expands the spectrum of mitochondrial cytopathies.
doi:10.4103/0972-2327.42942
PMCID: PMC2771970  PMID: 19893669
Chronic progressive external ophtalmoplegia; epilepsia partialis continua; mitochondrial dysfunction; Mitochondrial Encephalopathy with Ragged Red Fiber; MRI; periodic lateralized epileptiform discharges

Results 1-9 (9)