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Biomedical subjects

N Poungvarin

Publications and source records attributed to N Poungvarin.

At least 37 records · Page 2Linked to original sources

Botulinum A toxin treatment for blepharospasm and Meige syndrome: report of 100 patients.

One hundred patients (9.09 per cent) with blepharospasm from a grand total of 1,100 patients (at the Movement Disorders Clinic at Siriraj Hospital) who had been treated with botulinum A toxin (BTX-A) injection between 1989 and 1996 were analysed. The 100 patients comprised 65 females and 35 males with a female to male ratio of 1.86:1. Their mean age was 53.3 years (S.D. 12.03). Sixty patients had idiopathic blepharospasm, 31 patients were diagnosed with Meige syndrome (blepharospasm plus oromandibular dystonia) and nine patients with segmental dystonia (Meige syndrome plus cervical dystonia). The mean duration of suffering was 39.22 months (S.D. 44.83). Each patient received 30-50 IU of BTX-A injections according to the standard Siriraj injection pattern. Nine patients were lost to follow-up. The results of BTX-A injection were classified as: excellent result (an improvement of more than 75 per cent) in 83.51 per cent; a good response (an improvement of 50-75 per cent) in 13.19 per cent; a moderate response (an improvement of 25-50 per cent) in 2.20 per cent; and minimal or no response in 1.10 per cent. The complications of BTX-A injection were transient minimal ptosis (9.89 per cent), transient double vision (1.10 per cent) and excessive lacrimation (1.10 per cent). The efficacy of BTX-A injection lasted one to two months in 1.10 per cent, two to three months in 23.08 per cent, three to four months in 45.05 per cent, four to five months in 16.48 per cent, five to six months in 4.40 per cent and more than six months in 9.89 per cent. Botulinum A toxin injection is a simple and effective out-patient treatment for patients with blepharospasm, causing no systemic side-effects and minor transient local complications. The only drawback of this treatment is its high cost (100 IU cost 300 US dollars).

Adolescent↗

An open label trial of pergolide in Thai patients with Parkinson's disease.

Fifteen Thai patients with Parkinson's disease (7 females, 8 males) were enrolled in an open label trial of pergolide (a new dopamine agonist) to evaluate its safety and efficacy. Inpatients and outpatients from Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand from 1992 to 1994 were included in the study with a total duration of 18 weeks. Both de novo patients and patients who were being treated with levodopa without dopamine agonist and were obtaining a less than optimal response at both visit 1 and visit 2 were all enrolled in this study. At entry into the study, 3 patients had Hoehn and Yahr stage I, 7 patients at stage II, 3 patients at stage III, and 2 patients at stage IV. Pergolide dosage was gradually built up until an optimal dosage was achieved. The average dose of pergolide during the study was 0.94 mg/day (range 0.075 to 8 mg/day). All patients completed the study and no patients dropped out. Two patients (13.33 per cent) experienced nausea (on 0.4 mg/day and 0.075 mg/day), two patients (13.33 per cent) experienced sleepiness (0.50 mg/day and 0.075 mg/day) and one patient (6.67 per cent) unsteadiness on walking (0.50 mg/day). There was one patient who required pergolide up to 8 mg/day which is higher than the recommended dosage (5 mg/day) but this patient experienced no adverse effects and his disabled dyskinesic was abolished. Our study demonstrated the good toleration and efficacy of pergolide treatment for Thai patients with Parkinson's disease. This new dopamine agonist stimulates both D1 and D2 receptors in comparison to other dopamine agonists (bromocriptine and lisuride) which stimulate only D2 receptors.

Aged↗

Dementia in Thai patients with Parkinson's disease: analysis of 132 patients.

A cross-sectional design study was done at Siriraj Hospital from 1990 to 1994 to determine the prevalence of dementia in Thai patients with Parkinson's disease. There were 81 males and 51 females with a male to female sex ratio of 1.59:1. One hundred and thirty two patients with Parkinson's disease were enrolled in this study. The mean duration of illness was 3.5 (S.D. 3.53) years. All patients were screened for depression by using the Thai Geriatric Depression Scale and patients with Alzheimer's disease or stroke were excluded from the study. The Thai Mental State Examination (TMSE) is a standard test used in this study to identify dementia in Thai patients with Parkinson's disease. Thirty four patients (25.76 per cent) scored TMSE below 23 points (cut-off point for dementia) and were considered as having dementia. Twenty four patients (18.18 per cent) scored below 20 points and were considered as having severe dementia, 12 patients (9.09 per cent) scored between 20-22 points (classified as mild to moderate dementia). Thirty seven patients (28.03 per cent) were in the borderline group as they had TMSE scores between 23-25 points. Fifty nine patients (44.70 per cent) were determined as nondemented as their TMSE scores were over 25 points. The items of cognitive function tests in TMSE which were severely impaired in the demented group were recall, attention and calculation whereas registration was relatively unaffected. Orientation and language were moderately impaired in the demented group. Dementia in Thai patients with Parkinson's disease is a significant problem for long term care of patients because of its high prevalence (one-fourth of all patients) and nearly one-fifth had severe dementia. Prompt recognition and proper management of dementia are required for family members and medical personnel to handle patients with Parkinson's disease.

Aged↗

Treatment of various movement disorders with botulinum A toxin injection: an experience of 900 patients.

A prospective open study of botulinum toxin A treatment for patients with various movement disorders at Siriraj Hospital, Mahidol University was analysed to evaluate its efficacy. The grand total of 900 patients comprised of a) 592 patients (65.78 per cent) with hemifacial spasm; b) 92 patients (10.22 per cent) with occupational cramp; c) 79 patients (8.78 per cent) with blepharospasm and Meige syndrome; d) 72 patients (8.00 per cent) with spasmodic torticollis; e) 19 patients (2.11 per cent) with hemidystonia and generalised dystonia; f) 11 patients (1.22 per cent) with spasmodic dysphonia; g) 10 patients (1.11 per cent) with spastic hemiparesis; and h) 25 patients (2.78 per cent) with miscellaneous group (i.e. tics, Gilles de la Tourette, facial myokimia, benign fasciculation, etc.). The results of treatment for hemifacial spasm were classified as excellent in 486 patients (82.09 per cent), moderate improvement in 60 patients (10.14 per cent), mild improvement in 39 patients (6.59 per cent) and no improvement or worse in 7 patients (1.18 per cent). There were complications of mild transient facial weakness in 50 patients (8.45 per cent) and mild ptosis in 12 patients (2.02 per cent). The effect of botulinum toxin treatment lasted 3-6 months. In occupational cramp and spasmodic torticollis the good response rate was around two-thirds of all patients, whereas, blephalospasm, spasmodic dysphonia, spastic hemiparesis and tics responsed in 79-88 per cent of the patients. Botulinum toxin A injection is thus a simple, safe, and effective out-patient treatment for patients with various kinds of movement disorders but it is a costly therapy.

Botulinum Toxins↗

Dementia in Thai stroke survivors: analysis of 212 patients.

A cross-sectional designed study was done at Siriraj Hospital from 1990 to 1994 to determine the prevalence of dementia in Thai stroke survivors. Two hundred and twelve Thai stroke (both hemorrhage and infarction) survivors (132 males and 80 females) were enrolled in this study. The mean age of the study group was 62.78 (S.D. 11.12) years with the mean duration of stroke suffering of 2.09 (S.D. 2.74) years. All patients were screened for depression by using Thai Geriatric Depression Scale and patients with Alzheimer disease were excluded from the study. The Thai Mental State Examination (TMSE) is a standard test used in this study to identify stroke patients with dementia. Seventy stroke patients (33.02 per cent) scored below 23 points (cut-off point for dementia) and considered as dementia. Forty patients (18.88 per cent) scored below 20 points and were considered as having severe dementia, 30 patients (14.15 per cent) scored between 20-22 points (classified as mild to moderate dementia). Fifty-eight patients (27.36 per cent) were in the borderline group as they had TMSE scores between 23-25 points. Eighty four patients (39.62 per cent) of stroke survivors were determined as nondemented as their TMSE scores were over 25 points. The items of cognitive function tests in TMSE which were severely impaired in demented group were recall and calculation whereas registration and attention were relatively unaffected. Orientation and language were moderately impaired in the demented group.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebrovascular Disorders↗

Autosomal dominant cerebellar ataxia with dementia: evidence for a fourth disease locus.

The autosomal dominant cerebellar ataxias have proved particularly difficult to classify due to the lack of phenotypic concordance both within and between families. Genetic heterogeneity has been established, and disease loci for spinal cerebellar ataxia have been assigned to chromosomes 6 (SCA1), 12 (SCA2) and 14 (Machado Joseph disease (MJD)). Genetic analysis performed on a large Thai kindred with autosomal dominant cerebellar ataxia, in which frontal lobe signs and dementia are commonly observed in affected family members, exclude linkage to the SCA1, SCA2 and MJD loci. This demonstrates that mutation in at least one further locus can cause spinal cerebellar ataxia, indicating the need for caution in the use of markers for predictive testing or prenatal diagnosis these disorders.

Chromosome Mapping↗

Botulinum A toxin treatment in spasmodic torticollis: report of 56 patients.

Botulinum A toxin injection is the most recent and effective treatment of various movement disorders especially focal dystonia. Spasmodic torticollis is one focal dystonia which responds poorly to both medication and surgery. Botulinum A toxin injection has been adopted as a treatment procedure at the Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand since 1989 (before the American Food and Drug Administration approval) as a research protocal for Thai patients. This report is the first ever study of this treatment for Thai patients with spasmodic torticollis. Fifty six spasmodic torticollis patients who had been treated with botulinum A toxin injection at the Movement Disorder Clinic, Siriraj Hospital were analysed. Thirty six patients were male and the male to female ratio was 1.8:1. Most of the patients (76.79 per cent) were aged between 20-49 years and half of them were from Bangkok. Twelve patients (21.43 per cent) were classified as simple torticollis, 35 patients (62.5 per cent) were combined torticollis, 7 patients (12.5 per cent) were retrocollis, and 2 patients (3.57 per cent) were lateral collis. Three patients had generalised dystonia and 2 patients had segmental dystonia. Duration of suffering in each patient ranged from 1 month to 25 years with the mean duration of 3.70 (S.D. 5.09) years. Only four patients (7.14 per cent) refused botulinum A toxin injection due to their mild symptoms. The remaining 52 patients were given botulinum A toxin injection of 30-120 international units into the most overactive group of muscles which were responsible for abnormal neck posture (mainly sternocleidomastoid and splenius capitis). Eight patients (15.38 per cent) were lost to follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Hemifacial spasm: an electrophysiological evidence of facial motorneurons hyperexcitability.

Twenty patients with primary hemifacial spasm and ten patients with Parkinson's disease were studied by means of paired stimuli blink reflex. The second stimuli were delivered at a varying time interval between 50-900 ms. The R2 response's absolute refractory periods were compared between patients and twenty one age-matched normal controls as well as between hemifacial spasm and Parkinson's disease. In normal subjects, the mean absolute refractory period was found to be 271.42 +/- 64.36 ms, in hemifacial spasm 160.00 +/- 50.62 ms and in Parkinson's disease 157.14 +/- 53.45 ms. The absolute refractory periods obtained from patients were significantly shorter than in normal controls (p < 0.05). However the values between patients with hemifacial spasm and Parkinson's disease were not (p > 0.05). This result strongly supports bilateral facial motoneurons hyperexcitability as the underlying mechanism of hemifacial spasm.

Adult↗

A comparative study of coumadin and aspirin for primary cardioembolic stroke and thromboembolic preventions of chronic rheumatic mitral stenosis with atrial fibrillation.

The comparative study of the efficacy of coumadin and aspirin in primary cardioembolic stroke prevention of chronic rheumatic heart disease (mitral stenosis) with atrial fibrillation was conducted at Siriraj Hospital, Mahidol University, Bangkok, Thailand. Seventy-nine patients were enrolled in the trial. Allocation of patients into coumadin or aspirin groups depended upon the patients' choice. Nineteen patients were given coumadin at the adjusted dosage to maintain the therapeutic range of International Normalised Ratio between 1.5-3. Sixty patients were given aspirin at the fixed dosage of 75 mg per day. Six patients were lost to follow-up over the 3 yr period; four in the aspirin group and 2 in the coumadin group. There were three patients with nonfatal cardioembolic stroke in the aspirin group but none in the coumadin group after three years of follow-up. Six patients had mitral valve replacement during the study (i.e. three patients in each group). There were complications in 12 patients, 10 in the aspirin (16.6 per cent) and 2 in the coumadin (10.5 per cent) group. The complications in coumadin group were minor bleeding over the thigh in one patient and generalised ecchymosis over the whole body in one other. In the aspirin group, the complication was gastrointestional symptoms, mainly epigastric pain, but no frank bleeding was observed. Primary prevention of cardioembolic stroke in chronic rheumatic heart disease was found to be more effective with coumadin than aspirin. Our study does not support the use of aspirin in primary prevention of cardiac embolism in chronic rheumatic heart disease.

Adolescent↗

Serum lipid, lipoprotein-cholesterol and apolipoproteins A-I and B of smoking and non-smoking males.

Cigarette smoking is an important risk factor for coronary heart disease, stroke and peripheral vascular disease. Accordingly, we measured the serum lipid, lipoprotein-cholesterol and apolipoprotein A-I, B in 128 cigarette smoking males and 67 non-smoking males. The total cholesterol and LDL-cholesterol in smokers and non-smokers showed no statistical significance. The smokers had significantly higher serum triglyceride and VLDL-cholesterol levels (P < 0.001), but significantly lower HDL-cholesterol levels (P < 0.05) than non-smokers. The apolipoprotein A-I was significantly lower and apolipoprotein B was significantly higher in smokers than non-smokers (P < 0.001). Levels of lipid, lipoprotein-cholesterol were related to the number of cigarettes smoked per day. Triglyceride and VLDL-cholesterol levels were significantly higher in those who smoked > 20 cigarettes/day compared with those who smoked 10-20, < 10 cigarettes/day and non-smokers (P < 0.05). Those who smoked > 20 cigarettes/day had significantly lower HDL-cholesterol than those of non-smokers (P < 0.05). All three groups of smokers had significantly lower apolipoprotein A-I than non-smokers (P < 0.05), whereas, those who smoked > 20 cigarettes/day had significantly higher apolipoprotein B levels than those who smoked 10-20, < 10 cigarettes/day and non-smokers (P < 0.05).

Adult↗

Two hundred and fifty patients with hemifacial spasm treated with botulinum toxin injection.

Two hundred and fifty patients with hemifacial spasm from the Movement Disorder Clinic, at Siriraj Hospital have been treated with botulinum toxin injection since January 1989 as a collaborating research project with Smith-Kettlewell Eye Research Institute in San Francisco. Each patient received 30 units in four injection sites over the hyperkinetic facial muscles. There were 169 female and 81 male patients, the sex ratio of female to male was 2.1:1. The mean age of all patients was 50.2 +/- 12.6 years with the range of 22 to 78 years. The majority of patients had been suffering for 3-10 years. The results of botulinum toxin injection were classified as excellent in 81.2 per cent, moderate improvement 10.0 per cent, mild improvement 6.8 per cent and no improvement or worse in 2.0 per cent. There were complications of mild transient facial weakness in 44 patients (17.6%) mild ptosis in 7 patients (2.8%) and excessive lacrimation in 1 patient (0.4%). The effect of botulinum toxin treatment lasted for 3-6 months duration. Botulinum toxin injection is a simple and effective out-patient treatment for patients with hemifacial spasm with no systemic side effects and minor transient local complications.

Adult↗

Siriraj stroke score and validation study to distinguish supratentorial intracerebral haemorrhage from infarction.

OBJECTIVES: To develop a simple, reliable, and safe diagnostic tool for acute stroke syndromes in a setting where computerised brain scanning was not readily available and to validate its accuracy with regard to pathological types of stroke. DESIGN: 13 clinical variables that potentially might differentiate supratentorial cerebral haemorrhage from infarction were recorded and tested by multivariate analysis in a prospective study of 174 patients with acute stroke. In developing the Siriraj stroke score stepwise discriminant analysis of the variables was followed by a linear discriminant equation to differentiate between supratentorial haemorrhage and infarction. The score obtained was validated against scores in 206 other patients with stroke, computerised brain scans being used for definitive diagnosis. SETTING: Siriraj Hospital Medical School, Mahidol University, Bangkok. SUBJECTS: Prospective study: 174 consecutive patients with acute supratentorial stroke syndrome (not subarachnoid haemorrhage) admitted to Siriraj Hospital during 1984-5; validation study: 206 patients admitted to Siriraj Hospital or another hospital for supratentorial intracerebral haemorrhage or infarction. RESULTS: The Siriraj stroke score was developed and calculated as (2.5 x level of consciousness) + (2 x vomiting) + (2 x headache) + (0.1 x diastolic blood pressure) - (3 x atheroma markers) - 12. A score above 1 indicates supratentorial intracerebral haemorrhage, while a score below -1 indicates infarction. The score between 1 and -1 represents an equivocal result needing a computerised brain scan or probability curve to verify the diagnosis. In the validation study of the Siriraj stroke score the diagnostic sensitivities of the score for cerebral haemorrhage and cerebral infarction were 89.3% and 93.2% respectively, with an overall predictive accuracy of 90.3%. CONCLUSION: The Siriraj stroke score is widely accepted and applied in hospitals throughout Thailand as a simple and reliable bedside method for diagnosing acute stroke.

Acute Disease↗

Clinical study on antithrombotic effects of ticlopidine in ischemic stroke.

The investigators conducted a clinical study on antithrombotic effectiveness in ischemic stroke at Siriraj Hospital Medical School, Mahidol University from May 1987 to May 1989. Twenty-nine patients, 16 males and 13 females were enrolled in the study. The ages of the patients ranged from 30-87 years with a mean age of 63 +/- 11 years. Ticlopidine (250 mg) could significantly inhibit platelet aggregation induced by ADP and collagen within 24 hours of drug administration. After 1 week to 6 months, only aggregation by ADP was still inhibited significantly without significant effects on fibrinolytic activity and prostacyclin. Hematocrit was significantly decreased at the 1st and 2nd month of treatment. Serious side effects were skin rash and severe headache while the other common ones were dizziness, and diarrhea but these effects disappeared without discontinuing the drug. Most patients who suffered from nausea, diarrhea and headache, had temporary elevated SGPT. It may be concluded that only half of the recommended dose of ticlopidine has inhibitory effects on both phases of ADP-induced aggregation without interfering with fibrinolytic activity and can maintain prostacyclin. However, it also possesses either serious or common side-effects. This drug, therefore, should be used with the awareness of the clinician.

Adult↗

Multifocal brain damage due to lacquer sniffing: the first case report of Thailand.

The first patient of multifocal brain damage due to lacquer sniffing was reported in Thailand. He was a 24-year-old man who had a 5 year history of lacquer sniffing (toluene abuse). He had difficulty in walking and had tremor for 1 year which became progressively worse and he was finally confined to bed. Examination revealed marked cerebellar signs of both upper and lower extremities with scanning speech. Five of his friends who were lacquer abusers also had similar symptoms and were confined to bed. Investigations showed marked atrophy of both cerebellar hemispheres, vermis, brainstem and less atrophy of both cerebral hemispheres in both computerized brain scanning and magnetic resonance imaging. Diffuse toxic demyelination of white matter and excess iron deposition over both thalami, basal ganglia and cerebral cortex were demonstrated on magnetic resonance imaging. Brainstem evoked response showed abnormal response of both sides. Nerve conduction velocity, electromyographic study and electroencephalogram were normal. Psychometric tests revealed dull normal or below average IQ-test of 82. He was admitted for 2 months with gradual recovery of neurological deficits. After six months of abstinence from lacquer and daily physical rehabilitation. This report of toluene abuse is not only public health problem but also reflects the socioeconomic status as well as political unawareness of this condition in the Thai community.

Adult↗

Writer's cramp: the experience with botulinum toxin injections in 25 patients.

Twenty-five writer's cramp patients have been attending the Movement Disorder Clinic at the Division of Neurology, Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok during three years period (between January 1988 - January 1991). There were 17 male subjects and the male to female sex ratio was 2.125:1. The mean age of the patient was 36.80 (SD 10.21) years with the range of 18-60 years. The mean duration of illness of all patients was 5.88 (SD 7.14) years with the range of 1 to 30 years. Eighteen patients (72.0%) were classified as simple writer's cramp and seven patients (28.0%) were dystonic writer's cramp. The mean age of the patients of both groups was not different while the duration of illness in the dystonic group was statistically significantly longer than the simple group, i.e. 12.0 (SD 12.1) versus 3.9 (SD 3.1) years. Fourteen patients (56%) had associated pain during writing and 6 patients (24%) had hand tremor. All patients were right handed and had a history of various pharmacological treatments without any consistent benefit. They included muscle relaxants, tranquillisers, antiepileptic drugs, and betablockers. Fourteen patients from 17 available history records (82.4%) had been spending at least 4-10 hours writing each day. Twenty-one patients (84%) had botulinum toxin injections, 40-80 international mouse units were given in 2-4 divided doses over the overactive forearm muscles observed during writing without the electromyographic glidance. There was no loss to the follow-up. Fourteen of the 21 subjects (66.7%) showed definite improvement in hand writing, 4 patients (19.0%) improved minimally and 3 patients (14.3%) revealed no improvement. Arm pain in all 12 patients associated during writing was abolished after the injections. There were complications in 7 patients (33.3%) presented as transient finger drop (5 patients, 23.8%) and easily fatigued arm (2 patients, 9.5%). These preliminary results confirm that botulinum toxin injections is a successful treatment for many patients with writer's cramp without performing complex electromyographic recordings while the patients are writing. The constraints of this treatment are its high cost (i.e. 1 vial of 100 units costs 300 US dollars) and its benefit lasts for only 4-6 months.

Adolescent↗

Symptomatic vertebral haemangiomas: report of two cases.

The authors reported two cases of vertebral haemangiomas with spinal cord compression. The first case deteriorated during her pregnancy. Plain radiography of the thoracic spine gave the hint for the diagnosis and computed axial tomography showed the lesion. She was treated via surgical removal with minimal improvement of her paraparesis. The second case was diagnosed after operation because there was no abnormality over the relevant vertebra and the myelogram demonstrated an extradural mass lesion compressing the lower thoracic spinal cord. The patient improved satisfactorily after total removal of the tumour. Both surgical specimens were classical pictures of haemangiomas histologically.

Adult↗

Myotonia congenita, Klinefelter syndrome and primary hypoparathyroidism: the first report of the unusual combination of three rare diseases in the literature.

The first report of the rare combination of myotonia congenita, Klinefelter syndrome and primary hypoparathyroidism was reported. The patient was a 21-year-old man who presented with stiffness of the muscles for 12 years and a history of generalised convulsion for 8 years. His school studies declined gradually and his secondary schooling was interrupted. Examination revealed a muscular young man with myotonic percussion over the muscles of the body as well as the myotonic lid lag. Chvostek's sign was positive and his serum calcium level was very low whereas the serum phosphate was high. Symmetrical and extensive calcification of the brain parenchyma was demonstrated in the CT scan. His secondary sexual characteristics were not well developed and his testes were very small. Chromosome study confirmed the diagnosis of Klinefelter syndrome by the identification of 47 XXY chromosome. He was prescribed calcium replacement and the anticonvulsant was withheld with no more episodes of seizure.

Adult↗

Motor neurone disease in Thailand: the clinical aspects of 77 patients.

This is the first large scale case series of motor neurone disease (MND) in Thailand. Seventy-seven patients were identified between 1978 and 1984 at Siriraj Hospital Medical School, Bangkok, Thailand. Fifty-five patients were male (71.43%) and the mean age of the patients was 51.55 (SD 14.26) years with the range of 17 to 78 years. Clinical classification of MND was categorized as progressive bulbar palsy (PBP), 26 patients (33.77%); amyotrophic lateral sclerosis (ALS), 42 patients (54.54%); and progressive spinal atrophy (PSA), 9 patients (11.69%). The mean age of PBP, ALS and PSA were in the order of 57.61 (SD 12.09), 52.81 (SD 11.18), and 28.11 (SD 9.44) years. Progressive spinal atrophy group was younger than PBP and ALS groups significantly at the P-value less than 0.05 by analysis of variance and Duncan tests. Fifty-three patients (72.60%) were resident in Bangkok and the central part of Thailand. The main presenting symptoms were wasting of the small muscles of both hands, leg weakness, and speech and/or swallowing difficulties. These symptoms were found in 62 patients (81.58%). Nearly half of the patients (48.68%) came to our care within six months of onset, 22.8 per cent presented with asymmetry of motor wasting, while limb and trunk fasciculation was seen in 73.61 per cent. Dysarthria, dysphagia and tongue fasciculation were recorded as 51.32, 48.68, 60.53 per cent respectively. Exaggerated deep tendon reflexes were noted as 65.79 and 80.26 per cent over the upper and lower limbs, while Babinski sign was elicited in only 23.3 per cent of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗