Hoover's sign: Clinical relevance in Neurology.

Hoover's sign was described by Dr. Charles Franklin Hoover more than 100 years back to differentiate between the organic and functional weakness of pyramidal origin. This test is usually performed in the lower limbs and is valuable when on bedside one is not sure about the nature of hemiparesis. A subject with hemiparesis of organic cause while asked to flex the hip of normal leg against resistance will not exert pressure on the hand of examiner placed under the heel on the affected side while in hysterical weakness heightened pressure will be felt on the examiner's hand. The presumed genesis of this sign could be the crossed extensor reflex or the principle of synergistic contraction. It is a useful clinical test in differentiating functional and organic paresis with moderate sensitivity (63%) and high specificity (100%), but there are some limitations which should be kept in mind while evaluating a patient.

Pregnancy and puerperium-related strokes in Asian women.

BACKGROUND: Despite an increased risk of stroke in pregnancy and puerperium, the overall incidence of the condition in this population is low. Therefore, there is limited data pertaining to these patients particularly from Asian countries. Our objective was to describe the risk factors and outcomes of 110 pregnancy-related ischemic strokes from 5 Asian countries. METHODS: Data were collected by retrospective chart review in most cases and prospectively in the rest. Inclusion criteria for this subanalysis were women, pregnant or within 1-month postpartum, presenting to the study center with acute ischemic stroke (arterial or venous) confirmed by neuroimaging. Intracranial hemorrhages other than the ones associated with cerebral venous thrombosis or hemorrhagic infarct were excluded. Risk factors were diagnosed based on already published criteria. Outcomes were measured using modified Rankin score. Statistical analysis was done using Statistical Package for Social Sciences version 19.0. RESULTS: In all, 110 women with mean age of 27.94 years presented with pregnancy-related ischemic strokes; 58.2% of the strokes occurred postpartum and 49.1% were secondary to cerebral venous thrombosis. Venous strokes were significantly more likely to occur postpartum compared with arterial strokes (P=.01), to have abnormal "hypercoagulable panel result on admission" (P<.001), less likely to have traditional stroke risk factors (P<.001), to have hemorrhagic conversion of stroke (P<.001), and to have lesser stroke severity and better functional outcome at 3 months (P<.001 for each). CONCLUSION: Cerebral venous thrombosis is a significant contributor to pregnancy-related strokes in Asian women. Both traditional and pregnancy-specific risk factors should be addressed to control ischemic stroke risk in these women.

Predictors of cerebral venous thrombosis and arterial ischemic stroke in young Asian women.

The management and outcome of cerebral venous thrombosis (CVT) may be different from that of arterial ischemic stroke (AIS). Clinically differentiating the 2 diseases on clinical grounds may be difficult. The main objective of this study was to identify predictors differentiating CVT from AIS in a large cohort of young Asian women, based on risk factors and investigations. Twelve centers in 8 Asian countries participated. Women aged 15-45 years were included if they had a diagnosis of first-ever symptomatic AIS or CVT confirmed by brain computed tomography scan or magnetic resonance imaging/magnetic resonance venography. Patients with head trauma, cerebral contusions, intracranial hemorrhage, and subarachnoid or subdural hemorrhage were excluded. Data, including demographic data, risk factor assessment, neuroimaging studies, blood tests, and cardiac studies, were collected by retrospective and then prospective chart review between January 2001 and July 2008. Outcome was based on the modified Rankin Scale (mRS) score at admission, discharge, and latest follow-up. A total of 958 patients (204 with CVT and 754 with AIS) were included in the study. Age under 36 years, anemia, pregnancy or postpartum state, and presence of hemorrhagic infarcts on computed tomography scan or magnetic resonance imaging were significant predictors of CVT on univariate analysis. Age over 36 years, diabetes, hypertension, dyslipidemia, recent myocardial infarction, electrocardiogram abnormalities, and blood glucose level >150 mg/dL were strong predictors of AIS. On multivariate analysis, postpartum state and hemorrhagic infarct were the strongest predictors of CVT (P < .001). Mortality was comparable in the 2 patient groups. Prognosis was significantly better for patients with CVT than for those with AIS (mRS score 0-2, 74% v 46%; P < .001). There was no difference in outcome between patients with obstetric and nonobstetric CVT. Our data indicate that in young Asian women, predictors of CVT differ from those for AIS. These findings could be useful in the early identification and diagnosis of patients with CVT.

Ischemic stroke in young Asian women: risk factors, subtypes and outcome.

BACKGROUND AND OBJECTIVE: The objective of our study was to describe risk factors, mechanisms and outcome of young Asian women with ischemic stroke. METHODS: Twelve tertiary-care centers in 8 Asian countries participated. Women aged 15-45 years were included if they had an ischemic stroke supported by neuroimaging. Data on age, risk factor history, stroke mechanism and discharge status were collected. RESULTS: A total of 958 subjects were included, their mean age was 34 years. Large-vessel thrombosis comprised 24%, cerebral venous thrombosis 21%, cardioembolism 19% and small-vessel thrombosis 15%. The stroke risk factors included hypertension (29%), diabetes (14%), pregnancy (11%), valvular heart disease (10%) and cigarette smoking (3%). Anemia was found in 42%, and mortality was 4%; at discharge, 17% had modified Rankin score (mRS) >4 and 83% mRS 0-3. CONCLUSION: Unlike among Caucasians, large-vessel thrombosis, cerebral venous thrombosis and cardioembolism are common among young Asian women with stroke. A high proportion are pregnancy-related. More studies are needed.

Carbimazole induced ANCA positive vasculitis.

Anti-thyroid drugs, like carbimazole and propylthiouracil (PTU) are commonly prescribed for the treatment of hyperthyroidism. One should be aware of the side effects of antithyroid medications. Antineutrophil cytoplasmic antibody (ANCA)--associated vasculitis is a potentially life-threatening adverse effect of antithyroidmedications. We report a patient with Graves' disease who developed ANCA positive carbimazole induced vasculitis. The episode was characterized by a vasculitic skin rash associated with large joint arthritis, pyrexia and parotiditis but no renal or pulmonary involvement. He was referred to us for neurological evaluation because he had difficulty in getting up from squatting position and was suspected to have myositis. Carbimazole and methimazole have a lower incidence of reported ANCA positive side effects than PUT. To the best of our knowledge this is the first ANCA positive carbimazole induced vasculitis case reported from India.

Granulomatous hypophysitis--an interesting and rare cause mimicking pituitary mass.

Idiopathic granulomatous hypophysitis is a rare entity. The usual clinical presentation is that of an expanding mass lesion with varying degree of hypopituitarism. This patient described presented to us with severe headache along with panhypopituitarism and post-operative diabetes insipidus.

Epilepsy in India: nuptiality behaviour and fertility.

PURPOSE: The study was conducted to assess the marital status and fertility in adults with epilepsy. METHODS: The study group consisted of 240 subjects with epilepsy aged 15 and above, Unmarried males (m) 55, unmarried females (f) 73 and married males 48, married females 64. Data was collected according to standard protocol regarding age, sex, age at marriage and onset of seizure, effect of epilepsy on marriage, type of seizure and control, fertility pattern and abortions. The study group was followed up for 30 months. The data was compared with latest available state and national data using Student's 't'-test, chi square, Fisher Z-test. RESULTS: The mean age of married subjects was 26.19 + 6.2 years and of the unmarried was 24.94 +/- 7.1 years. People with epilepsy had significantly (p < 0.05) higher mean age at marriage, significantly (p < 0.05) lower rate of marriage (m 46.6% f 46.72%), significantly (p < 0.05) higher divorce rate and withheld marriage. Females compared to males had higher divorce rate (f 5.84%) and withheld marriage (m 5.45% f 10.96%). The marriage rate of subjects with onset of epilepsy in the first decade (m 38.71% f 34.09%) and second decade (m 33.33% f 35.42%) was significantly lower (p < 0.05) than that of subjects with age at onset of epilepsy greater than 20 years (m 66.67% f 71.11%). Subjects with epilepsy not in remission had significantly (p < 0.05) higher mean age at marriage, lower marriage rate and lower fertility per person-year of marriage than those in remission. Majority (95.54%) did not disclose epilepsy before marriage. Total fertility rate (TFR) in males (4.56) and females (5.45) with epilepsy was comparable to general population. Abortions per pregnancy were 4.76%. CONCLUSION: Subjects with epilepsy had lower marriage prevalence rate, delayed marriage (especially females), withheld marriage and higher divorce rate compared to general population. Marriage rate was lower in people with age at onset of epilepsy less than 20 years and in whom seizures were not in remission. Majority of people with epilepsy did not disclose epilepsy before marriage. Though fertility was not affected in people with epilepsy as compared to general population, males had lower fertility than females.

Clinical and surface EMG characteristics of valproate induced tremors.

PURPOSE: To describe the Clinical and Surface EMG characteristics of Valproate induced Tremors. Sodium Valproate is known to produce tremors as a side effect. We analyzed clinical and surface electromyographic characteristics of tremors in epileptic subjects who presented with tremors while on sodium valproate. METHODS: Neurological examination and surface electromyographic recordings were performed on 20 subjects with sodium valproate induced tremors. Surface electromyographic tremerogram was correlated with clinical presentation of tremors. RESULTS: This study revealed sodium valproate induced tremors involving primarily upper limbs symmetrically. In the present study, all patients presented with action postural tremors except two, who had tremors in the resting position as well. None of these patients had tremors in goal-directed position. The tremors typically were of high frequency (mean 10 Hz), low amplitude, short burst duration and synchronous burst pattern (Synchronous EMG activity in the agonist and antagonistic muscles). CONCLUSIONS: Frequency, amplitude, burst duration and burst pattern of sodium valproate induced tremors resembles benign essential tremors on surface electromyographic recordings.

Adrenomyeloneuropathy.

Adrenomyeloneuropathy is a rare X-linked inherited disorder of peroxisomes characterized by the accumulation of very long chain fatty acids (VLCFA) in the central and peripheral nervous system, adrenal glands and testis leading to the dysfunction of these organs and systems. The phenotypic expression is highly variable, childhood cerebral adrenoleukodystrophy and adrenomyeloneuropathy being the main variants. Here we report a case of adrenomyeloneuropathy presenting initially as Addison's disease, which remained undiagnosed for many years with many rare features. We could not locate any other publication on this subject in the indexed journals of literature published from India.

IgA monoclonal gammopathy of undetermined significance with peripheral neuropathy.

Monoclonal gammopathy of undetermined significance (MGUS) and peripheral neuropathy may be causally linked. In most cases, the M-protein is of IgG or IgM type. Peripheral neuropathy associated with IgA MGUS is uncommon, and there are limited reports. Here, we report a case of a 55-year-old male who was diagnosed to have symmetrical sensorimotor peripheral neuropathy associated with IgA MGUS with deposits of IgA-monoclonal protein in the myelin sheath.

Cervical mass lesion presenting as reflex sympathetic dystrophy of the hand.

A case with multiple small joint swelling and skin dystrophic changes in the right hand was initially treated as seronegative non-specific arthritis. Later, a triple phase bone scan indicated reflex sympathetic dystrophy (RSD) and the primary lesion was shown on MRI scan to be a cervical cord mass with associated sub-acute haematoma. The case underscores the need to consider RSD in the differential diagnosis of such conditions and the importance of a detailed neurological examination in RSD with no local causative factor.

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is a peripheral neuropathy caused by peripheral nerve inflammation probably due to autoimmunity and would be expected to benefit from corticosteroids. Non-randomised studies suggest that corticosteroids are often beneficial. OBJECTIVES: To evaluate the efficacy of corticosteroids for treating chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Group register for randomised trials of corticosteroids treatment for chronic inflammatory demyelinating polyradiculoneuropathy and enquiry from subject experts. SELECTION CRITERIA: Types of studies: All randomised or quasi-randomised trials Types of Participants: All patients with chronic inflammatory demyelinating polyradiculoneuropathy who were diagnosed by an internationally accepted definition. Types of interventions: Treatment with any form of corticosteroids or adrenocorticotropic hormone. Types of outcome measures: PRIMARY OUTCOME MEASURE: Change in disability 12 weeks after randomisation. SECONDARY OUTCOME MEASURES: 1. Change in impairment 12 weeks after randomisation. 2. Change in maximum motor nerve conduction velocity or compound muscle action potential amplitude after 12 weeks. 3. Side effects of corticosteroids. DATA COLLECTION AND ANALYSIS: One author extracted the data and the other checked them. MAIN RESULTS: We identified one randomised controlled trial, an open study in which 19 corticosteroids treated patients showed more improvement in impairment than 16 untreated controls after 12 weeks. Experience from large non-randomised studies suggests that steroids are beneficial. REVIEWER'S CONCLUSIONS: A single randomised controlled trial with 35 participants provided weak evidence to support the conclusion from non-randomised studies that oral corticosteroids reduce impairment in chronic inflammatory demyelinating polyradiculoneuropathy. Corticosteroids are known to have serious long term side effects. The long term risk and benefits have not been adequately studied.

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune peripheral neuropathy and would be expected to benefit from corticosteroids. Non-randomised studies suggest that corticosteroids are often beneficial. OBJECTIVES: To evaluate the efficacy of corticosteroids for treating chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Group register for randomised trials of corticosteroids treatment for chronic inflammatory demyelinating polyradiculoneuropathy and enquiry from subject experts. SELECTION CRITERIA: Types of studies: All randomised or quasi-randomised trials Types of participants: All patients with chronic inflammatory demyelinating polyradiculoneuropathy who were diagnosed by an internationally accepted definition. Types of interventions: Treatment with any form of corticosteroid or adrenocorticotropic hormone. Types of outcome measures: PRIMARY OUTCOME MEASURE: Change in disability 12 weeks after randomisation. SECONDARY OUTCOME MEASURES: 1. Change in impairment 12 weeks after randomisation. 2. Change in maximum motor nerve conduction velocity or compound muscle action potential amplitude after 12 weeks. 3. Side effects of corticosteroids. DATA COLLECTION AND ANALYSIS: One author extracted the data and the other checked them. MAIN RESULTS: We identified one randomised controlled trial, an open study in which 19 corticosteroid treated patients showed more improvement in impairment than 16 untreated controls after 12 weeks. Experience from large non-randomised studies suggests that steroids are beneficial. REVIEWER'S CONCLUSIONS: A single randomised controlled trial with 35 participants provided weak evidence to support the common opinion derived from non-randomised studies that oral corticosteroids reduce impairment in chronic inflammatory demyelinating polyradiculoneuropathy. Corticosteroids are known to have serious long term side effects. The long term risks and benefits have not been adequately studied.

Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an uncommon progressive or relapsing paralysing disease caused by inflammation of the peripheral nerves. If the hypothesis that it is due to autoimmunity is correct, removal of autoantibodies in the blood by plasma exchange should be beneficial. OBJECTIVES: To evaluate the efficacy of plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: We searched the Neuromuscular Disease Group Register (December 2003), and MEDLINE (January 1966 to January 2003), EMBASE (January 1980 to January 2003), CINAHL (January 1982 to December 2002) and LILACS (January 1982 to January 2003). We also scrutinised the bibliographies of the trials, and contacted the trial authors and other disease experts. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials in participants of any age comparing plasma exchange with sham treatment or no treatment. A trial showing no significant difference in the benefit from plasma exchange with intravenous immunoglobulin has been included in a separate Cochrane review. DATA COLLECTION AND ANALYSIS: Two authors selected the trials, extracted the data and assessed methodological quality independently. Where possible data were combined according to the methods of the Cochrane Neuromuscular Disease Review Group. PRIMARY OUTCOME MEASURE: one crossover trial including 18 participants showed 2 (95% confidence interval (CI) 0.8 to 3.0) points more improvement after four weeks in an 11-point disability scale with plasma exchange (10 exchanges over four weeks) than with sham exchange. Rapid deterioration after plasma exchange occurred in eight of 12 who had improved. SECONDARY OUTCOME MEASURES: when the results of this trial and another with 29 participants treated in a parallel group design trial were combined, there were 31 points (95% CI 16 to 45) more improvement in an impairment scale after plasma exchange (six exchanges over three weeks) than after sham exchange. There were significant improvements in both trials in an electrophysiological measure, the proximally evoked compound muscle action potential, after three or four weeks. Non-randomised evidence indicates that plasma exchange induces adverse events in 3% to 17% of procedures. These are sometimes serious. REVIEWERS' CONCLUSIONS: Evidence from two small trials showed that plasma exchange provides significant short-term benefit in about two-thirds of patients with chronic inflammatory demyelinating polyradiculoneuropathy but rapid deterioration may occur afterwards. Adverse events related to difficulty with venous access, use of citrate and haemodynamic changes are not uncommon. More research is needed to identify agents which will prolong the beneficial action of plasma exchange.

Epidermoid cyst in association with subcutaneous meningioma.

A 17-year-old female presented with a non-tender, firm and fixed swelling in the right frontal region. Skull x-rays revealed soft tissue shadow and mild hyperostosis of the underlying frontal bone. Histopathological examination after excision showed it to be a meningioma with an epidermoid cyst.

Clinical expression and EEG features of patients with juvenile myoclonic epilepsy (JME) from North India.

We aimed to characterize the clinical profile, EEG features and response to treatment of juvenile myoclonic epilepsy (JME) patients. We studied 103 JME probands with a standard protocol recording age of onset, type, frequency of seizures, EEG data, detailed family history and response to treatment in a superspeciality university hospital in New Delhi. The mean age of onset of disease was 14.01 +/- 3.14 years with a male to female ratio of 1.19 : 1. The myoclonic jerks were present in all the probands, generalized tonic-clonic seizures (GTCS) were present in 75.72% and 11.65% probands had absence seizures. The incidence of febrile convulsion (FC) was higher (9.7%) in our JME probands reflecting some ethnic variation or ascertainment bias. There was a considerable delay (of approximately 5.26 +/- 4.61 years) in the diagnosis of JME in our probands because most of the patients were referred from private physicians who were possibly not familiar with this epileptic syndrome in this part of the world or thought it was a milder variety of GTCS. The family history was positive in 25.24% JME probands among first- and second-degree relatives. An interictal EEG was found to be abnormal in 81 (78.64%) patients with the predominant abnormality being generalized polyspike and wave (PSW) discharges (39.80% probands). The majority of patients (80.58%) showed a good response to treatment with valproate alone. There was a subset of patients (11.65%) who required the addition of other antiepileptic drugs (AEDs) for control of GTCS: 7.76% of JME patients were diagnosed as cases of GTCS by private practitioners before they were registered in our study and their seizures were well controlled on other AEDs (without valproate) prescribed by the referring physicians (carbamazepine-4, phenytoin-2, clobazam-2). It is concluded that the clinical features and EEG data of JME probands were comparable to reports from other parts of the world except for the fact that the incidence of FC was higher in our JME patients. There was a delay in the diagnosis of JME due to unfamiliarity with the epileptic syndrome among private practitioners in this part of the world. There was a subset of JME patients who had complete seizure control on other AEDs besides valproate.

Clobazam monotherapy in drug naïve adult patients with epilepsy.

PURPOSE: Evaluation of the efficacy and side effects profile of Clobazam in a 24-week open-labelled trial involving 26 cases of drug naïve adult patients with epilepsy. METHODS: The study was an open labelled unicentre trial in which only drug naïve cases with epilepsy were included. A total of 26 cases were recruited. One case was dropped because he did not complete the desired follow up. Seizure type and frequency were recorded and follow up was done at 4, 8, 12, 18 and 24 weeks after initiation of therapy. The change in seizure severity, the dose of Clobazam required and development of side effects were recorded. RESULTS: The seizure types included GTCS (n=16), complex partial seizures (n=4), focal motor seizures with secondary generalisation (n=3) and juvenile myoclonic epilepsy (n=2). Out of 25 patients, 16 (64%) became seizure free, while five (20%) had >50% reduction in their seizure frequency. Thus, these 21 patients (84%) were considered to be well controlled. The commonest side effect seen was sedation, which was noted in 4 of the 25 patients (16%). However, in none of these four patients sedation was significant enough to warrant stoppage of therapy. Weight gain, gait ataxia, loss of short-term memory and breakthrough seizures were noted in one patient each. CONCLUSIONS: The efficacy of Clobazam coupled with the lack of significant side effects noted in our study makes it merit consideration as monotherapy in adult patients with epilepsy.

Neurological mitochondrial cytopathies.

The mitochondrial cytopathies are genetically and phenotypically heterogeneous group of disorders caused by structural and functional abnormalities in mitochondria. To the best of our knowledge, there are very few studies published from India till date. Selected and confirmed fourteen cases of neurological mitochondrial cytopathies with different clinical syndromes admitted between 1997 and 2000 are being reported. There were 8 male and 6 female patients. The mean age was 24.42+/-11.18 years (range 4-40 years). Twelve patients could be categorized into well-defined syndromes, while two belonged to undefined group. In the defined syndrome categories, three patients had MELAS (mitochondrial encephalopathy, lactic acidosis and stroke like episodes), three had MERRF (myoclonic epilepsy and ragged red fibre myopathy), three cases had KSS (Kearns-Sayre Syndrome) and three were diagnosed to be suffering from mitochondrial myopathy. In the uncategorized group, one case presented with paroxysmal kinesogenic dystonia and the other manifested with generalized chorea alone. Serum lactic acid level was significantly increased in all the patients (fasting 28.96+/-4.59 mg%, post exercise 41.02+/-4.93 mg%). Muscle biopsy was done in all cases. Succinic dehydrogenase staining of muscle tissue showed subsarcolemmal accumulation of mitochondria in 12 cases. Mitochondrial DNA study could be performed in one case only and it did not reveal any mutation at nucleotides 3243 and 8344. MRI brain showed multiple infarcts in MELAS, hyperintensities in putaminal areas in chorea and bilateral cerebellar atrophy in MERRF.

Antiphospholipid antibodies syndrome in 'Stroke in young'.

Antiphospholipid antibodies syndrome has emerged as an important entity responsible for stroke in young. Seven cases of young stroke (< 40 years of age) with mean age of 30.1 years (age range 25-39 years, 2 males and 5 females), who tested positive for antiphospholipid antibodies are being reported. All subjects had completed strokes. Six had arterial ischaemic and one patient had venous stroke. One patient suffered from four episodes, three ischaemic and one intracerebral haemorrhage. Two patients suffered from foetal loss. Generalised tonic clonic seizures occurred in three patients. Deep vein thrombosis was observed in one case. Thrombocytopenia was not observed in any case. All the patients had elevated anticardiolipin antibodies (aCL) IgM or IgG, while Lupus anticoagulant (LA) was elevated in 4 cases. Six cases belonged to primary antiphospholipid antibodies syndrome and one to lupus like illness. Oral anticoagulants were administered to maintain a high intensity international normalized ratio (INR). No recurrences were observed during a follow up period of 6-18 months.

Insulinoma presenting with dementia and hemiplegia.

A 23 year non-diabetic female presented with history of episodic alteration of sensorium, cognitive decline and right hemiparesis. Fasting blood glucose was found to be low on multiple occasions along with raised insulin levels. Abdominal CT scan and coeliac axis angiogram were normal. The diagnosis of insulinoma was confirmed on removal of the tumour on exploratory laprotomy. The patient was left with residual mild dementia and hemiparesis.