Juvenile myoclonic epilepsy(JME) of Janz is a syndrome of generalized age related epilepsy with increasing incidence and probable autosomal recessive trait. We have experienced 2 female patients who had awakening myoclonus in both arms, which had started in their early 10th. The patterns of seirures and electroencephalographic findings were consistent with those of JME which had been first described as impulsive petit mal by Janz. We report these cases with brief review of literatures.
Arm ; Epilepsy ; Female ; Humans ; Incidence ; Myoclonic Epilepsy, Juvenile* ; Myoclonus

The amplitude and latency of visual evoked potentials to pattern-reversal half-field stimulation were measured in 20 normal right-handed subjects to determine whether the individual contribution of right and left cerebral hemispheres is different as related to the generation of pattern-evoked potentials. The amplitude and latency of P1 from a lateral occipital electrode ipsilateral to the halffield stimulation were significantly greater with right half-field wtimulation. The amplitude and latency difference between both sides probably reflects asymmetries of human striate cortex.
Cerebrum ; Electrodes ; Evoked Potentials, Visual* ; Humans ; Visual Cortex

Electrodiagnostic studies were carried out in 31 patients with neurological complications of AHC, who were seen at Seoul National University Hospital from August 1981 to February 1982. Age of the patients ranged from 14 to 62 years. Both velocity and distal latency of motor and sensory conduction were normal in the nerves innervating the affected muscles. During the acute phase of motor paralysis, there was an absence of electrical activity in completely paralysed muscles. In partially denervated muscles, there were polyphasic motor unit potentials of normal duration and amplitrde on weak contraction and reduced interference on maximal effort. From the third or fourth week onwards, fibrillation potentials and positive sharp waves at rest were observed in paraspinal muscles in most cases. All the above findings were supporting the view that the principal site of involvement for the paralytic phenomena is at the level of the anterior horn cells or anterior roots.
Anterior Horn Cells ; Conjunctivitis, Acute Hemorrhagic* ; Humans ; Muscles ; Paralysis ; Paraspinal Muscles ; Seoul

Paraplegia following intrathecal methotrexate(MTX) instillation is a rare complication and 18 cases have been reported in the literature. It has not been reported m Korea. We recenty experienced a 56 year-old female with quadriplegia following intrathecal MTX instillation. So we report a case of paraplegia following intrathecal MTX instillation with review of the literature.
Female ; Humans ; Korea ; Methotrexate* ; Middle Aged ; Paraplegia* ; Quadriplegia

We performed 3 kinds of memory tests and cognitive evoked potential study, P300, in 13 patients with memory disturbance and 19 control free of neurologic symptoms or history of neurological impairment; to determine correlation of each memory test with P300 and possibility of clinical application of P300 as a quantitative test of cognitive function. Patients and control younger than 40 years of age were selected to minimize the aging effect on the cognitive function tests. Those who marked 30 points in Mini-mental state examination were chosen as control. 11 of 13 patients had brrain lesions including temporal lobe. Comparing with tests in control group, the declarative ant the procedural memory of patients were significantly impaired (p<0.05, p<0.025, respectively), and P300 latency was significantly prolonged (p<0.001). In control group, among declarative memory tests Rey-Osterreith complex figure test and enhanced cued recall had significant correlation with P300 latency (p<0.05, p<0.05, respectively), while the Tower of Toronto test which was known to evaluate precedural memory did not. In patient group there was no significant correlation between any kind of memory test and P300 latency. These results not only are consistent with previous studies which detected temporal lobe as the origin of P300 wave, but implicate that brain loci other than temporal lobe might originate P300 wave. To apply P300 as a quantitative test of cognitive function, further extensive studies using age and IQ matched control will be needed.
Aging ; Ants ; Brain ; Evoked Potentials* ; Humans ; Memory* ; Neurologic Manifestations ; Temporal Lobe

Adie's tonic pupil usually consists of mydriasis, defect in accommodation. Poor or absent light reflex, and tonic near reflex, It is usually unilateral, but rarelY observed bilaterally. The pupillary abnormalities often accompany areflexia, which is called Adie syndrome collechvely, Other autonomic dysfunction especially hypohidrosis. Are rarely reported in association with Adie syndrome. We recently experienced 2 cases of bilateral Adie's pupils in 2 women, one of whom had hypohidrosis, and the other had areflexia. So we report 2 cases with bilateral Adie's pupils with a review of literature.
Adie Syndrome ; Female ; Humans ; Hypohidrosis ; Mydriasis ; Reflex ; Tonic Pupil*

A 47-year-old man had suffered oscillopsia associated with palatal myoclonus for 10 years. High-field magnetic resonance imaging (MRI) revealed a cryptic vascular malformation within the "Guillain-Mollaret triangle" which was thought to be the responsible lesion.
Brain Stem/*abnormalities/pathology ; Humans ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Myoclonus/*etiology

Atlanto-axial instability in children with Down syndrome(trisomy 21) has become a wel! Known entity and it has been reported frequently since it was first reported over 25 years ago. However, symtomatic atlanto-axial dislocation associated with cord compression is relatively rare and it had seldom been reported. We recently experienced a 14-year-old mongoloid female with progressive quadriparesis, who have no prior cervical inflammation and we detected atlanto-axial dislocation with ossculum terminale and cord compression through radiological evaluations. We report a case of symptomatic atlanto-axial dislocation in Down syndrome with review.
Adolescent ; Child ; Dislocations* ; Down Syndrome* ; Female ; Humans ; Inflammation ; Quadriplegia

Twenty patient's capsular lesions were detected by high resolution brain computed tomograhpy (CT). Eighteen of them had systemic hypertension. Two patients had prior stroke episodes that were of the same nature. All of the patients had sudden onset of neurologic deficits. Their neurologic syndromes were pure motor stoke (10 cases), ataxic hemiparesis (3 cases), pure motor stroke with dysphasia (1 cases), pure sensory-motor stroke (3 cases), pure motor stroke with tremor, locked-in syndrome, and hemiparesis with latered consciousness, each 1 case respectively. The CT showed capsular infarcts of ovolid or round hypodense lesion that was smaller than 20mm in diameter and that was seen on only two scan sections. The sites of capsular infarcts were genu(5 cases), posterior limb (9 cases), genu-posterior limb (3 cases), genu-anterior limb (3 cases), and anterior limb (4 cases) respectively. Large capsulo-putamino-caudate infarcts, or giants lacunes (type 1 of Rascol et al ) are seen in 3 cases only in pure motor stroke ; Posterior limb capsular Iacunes (type 2 of Rascol et al), 6 cases in pure motor stroke, 3 cases in ataxic hemiparesis, 2 cases in pure sensory-motor stroke, 1 case in modified pure motor stroke with tremor : Anterior capsular lacunes (type 3 of Rascol et al), 1 case in pure motor stroke, 1 case in modified pure motor stroke with dysphasia, 1 case in modified pure motor stroke with memory and cognitive dysfunction. None of the capsular infarct caused mass effect. In five cases there was a relatively complete neurologic improvement, in 14 cases did the patients show mild residual neurologic deficit, and one patient expired. With the use of CT, the diagnosis of capsular infarcts has moderately increased.
Aphasia ; Brain ; Consciousness ; Diagnosis ; Extremities ; Humans ; Hypertension ; Memory ; Neurologic Manifestations ; Paresis ; Quadriplegia ; Stroke ; Tremor

The purpose of this study was to evaluate the efficiency of Magnetic Resonance Imaging (MRI) in the evaluation of patients with lacunar syndrome, to compare the sensitivity of MRI and that of brain Computed Tomography(CT) in the lacunar lesions. Thirty-nine cases of lacunar syndrome were pure sensory stroke(1 case), pure motor hemiparesis (12 cases), ataxic hemiparesis (5 cases), pure motor hemiparesis sparing face (2 case), pure motor hemiparesis with confusion (1 case), cerebellar ataxia with crossed 3rd nerve palsy (1 case), sensory-motor stroke(4 cases), lower basilar branch syndrome (6 cases), lateral medullary syndrome (3 cases), lateral pontomedullary syndrome (2 cases), locked-in syndrome (1 case), and miscellaneous (1 case). MRI detected small, deep lesions appropriate to lacunar syndrome in 34 cases (medulla, 3 cases; pons, 16 cases; midbrain, 3 cases : thalamus-internal capsule, 2 cases : internal capsule-basal ganglia, 6 cases; periventricular white matter, 3 cases) of the patients (34/39, 87.1%), and was diagnostically superior to CT in those cases (especially in brainstem lacunar lesions), where both studies were obtained concommitantly (MRI : CT=84.3% : 37.5%). MRI, when evailable, should replace CT as the diagnostic imaging procedure of choice in evaluation of lacunar syndrome.
Brain Stem ; Brain* ; Cerebellar Ataxia ; Diagnostic Imaging ; Ganglia ; Humans ; Lateral Medullary Syndrome ; Magnetic Resonance Imaging* ; Mesencephalon ; Paralysis ; Paresis ; Pons ; Quadriplegia ; Stroke, Lacunar*