Location of Primary Motor Cortex Function in Cerebral Migration Disorder

PURPOSE: The purpose of this study was to demonstrate by functional MRI (MRI) the location of the primarymotor cortex in patients with schizencephaly. MATERIALS AND METHODS: fMRI was performed in four patients withschizencephaly who complained of seizures;three were right handed and one was ambidex trous. Associated lesionswere agenesis of the corpus callosum in one patient and absence of the septum pellucidum in another. fMRI employedthe single sliced FLASH BOLD technique using a 1.5-T MR imager with a standard head coil, and was obtained in theaxial plane. Thirty consecutive images were obtained on finger movements of each hand were obtained;the motor taskconsisted of repetitive finger to thumb opposition. Percentage change in primary motor cortex signal intensity wascalculated, and ipsilateral activation index was compared betweenthe affected and unaffected hemispheres. RESULTS: Percentage change in signal intensity increase in the activated area of the unaffected hemisphere ranged from4.8% +/-0.9% to 9.2+/-1.2%(mean:5.6%+/-1.5%) of the baseline value. The ipsilateral activation index of the affectedhemisphere was 0-0.38 and that of the unaffected hemisphere was 15.4-Infinity;in patients with schizencephalysignificantly different(p<0.01). CONCLUSION: Our results suggest that increased activation in the unaffectedhemisphere reflect functional reorganization of the primary motor cortex.

Semiology and its Lateralizing Signs in Mesial Temporal Lobe Epilepsy

We analysed video tapes in 31 patients of 97 seizures who were evaluated for epilepsy surgery at AMC Neurology department between 1992 and 1994. All patients had epilepsy surgery such as temporal lobe resection or gamma knife radiosurgery. They were either seizure free or had a greater than 90% reduction in the number of seizures for at least 12 months following surgery. Oromandibular automatism (74.2%), behavioral arrest/staring (63.9%), and upper extremity automatisms (37 1%) were frequent ictal manifestations of mesial temporal lobe epilepsy in our patients, Initial non-versive head turning was observed in 49 events (ipsilateral 77.8%, contralateral 22.2%), while versive head turning was noticed in 25 events (ipsilateral 7.1%, contralateral 92.9%). Contralateral dystonic posture was observed in 47 events (48.5%). Vocalization was observed in 2 among 12 patients (16.7%) with non-dominant sided lesions, and 9 among 19 patients (47.4%) with dominant sided lesions. Dystonic posture was observed contralaterally (100%), hand automatism ipsilaterally (94.4%) and versive head/eye turning wart observed ipsilaterally (92.3%) in our patients. So we concluded that valuable lateralizing sign in mesial temporal lobe origin seizure are contralateral dystonic posture, ipsilateral hand automatism, and versive contralateral head/eye turning. And also turning and rounding sign may have possible lateralizing value.

Correlation of Icatal Scalp/Sphenoidal EEG Patterns and MRI Findings with Surgical Outcome in Temporal Lobe Epilepsy

BACKGROUND & OBJECTIVE: It has not been clear that the correlation between individual ictal scalp/sphenoidal EEG patterns and MRI findings or surgical outcome. So we investigated the correlation between the differences in individual ictal EEG patterns, MRI findings and surgical outcome. METHOD: Twenty nine temporal lobe epilepsy patients undergoing anterior temporal lobectomy with hippocampectomy in AMC since 1993 were evaluated. Ictal EEGs were classified as A(focal ictal onset) and B(lateralizing pattern) MRI findings were grouped as hippocampal atrophy (HA) with or without lateral temporal atrophy (LTA). RESULT: In group A(19), 7(37%) had HA with LTA on MRI and 12(63%) showed HA only. In group B(10), 8(80%) had HA with LTA and 2(20%) showed HA only. Among the 20 patients whose postoperative follow up period were more than 1 year, 11 cases(73%) of group A(IS) had seizure free outcome, whereas all (100%) of group B (5) had seizure free outcome. Eight (72.7%) out of 11 cases with HA only on MRI were seizure free outcome, whereas 8(89%) out of 9 cases with HA with LTA were seizure free after surgery. CONCLUSION: Focal ictal EEG pattern was more commonly noticed in HA only and lateralized Ictal EEG pattern was more commonly noticed in HA with LTA on MRI. Surgical outcome seems not to be directly correlated with the ictal EEG patterns or accompanied LTA on MRI in temporal lobe epilepsy.

Pathologic Analysis of 39 Cases of Epilepsy Surgery

Pharmacologic therapy is still the primary management for epilpsy; however, surgical treatment is a reasonable therapeutic option for patients suffering from medically intractable seizures, especially temporal lobe epilepsy having a documented unilateral epileptogenic area. Thirty nine patients with pharmaco-resistant complex partial seizures underwent anterior temporal lobectomy and hippocampectomy in 38 cases and frontal cortisectomy in one case. On pathological examination, hippocampal sclerosis was a predominent pathologic finding and was identified in 18 cases. Other non-neoplastic lesions consisted of 5 cases of vascular lesions(2 cavernous angiomas, 2 arteriovenous malformations and 1 angiomatosis), 3 cases of fibrous nodule, 2 cases of cicatrical changes of cerebral cortex, and 1 case of parasitic infection. Neoplasms including two cases of oligodendroglioma and one case of anaplastic astrocytoma were also noted. In seven cases, there was no detectable lesion on gross and microscopic examination. On post-operative follow-up, seizures were completely terminated in most cases(31 cases, 79%). The rest of the patients also displayed marked alleviation of symptoms. The seizures tended to recur more aften among the patients with neoplasm or no pathologically detectable lesion. In order to detect any minute pathological lesion, thorough gross and microcsopic examinations are considered to be essential.

Correlation between Preexcision ECoG Patterns and MRI Findings and Clinical Significance of Postexcision ECoG after Temporal Lobectomy

Role of intraoperative ECoG is uncertain and still controversial. We investigated the correlation of preexcision ECoG (pre-ECoG) patterns with MRI findings and the correlation of the presence or absence of residual spikes in postexcision ECoG (post-ECoG) with surgical outcome. We retrospectively studied 29 temporal lobectomized patients (follow-up period: > 16 mons). The ECoG was performed using two subdural strips on the mesiobasal and lateral temporal lobe. Topography and frequency of epileptiform discharges (ED) in pre-ECoG were analyzed and correlated with hippocampal atrophy only or hippocampal and lateral temporal atrophy in MRI. Among the 15 patients with hippocampal atrophy only, pre-ECoG showed mesial temporal dominant ED in 8 ( 53.3 %), diffuse ED in 3 ( 20.0 %) and lateral temporal dominant ED in 4 ( 26.7 %). In 14 patients with hippocampal and lateral temporal atrophy, mesial temporal dominant ED showed in 2 ( 14.3%), diffuse ED in 8 ( 57.1 %), and lateral temporal dominant ED in 4 ( 28.6%). Compared to patients with hippocampal atrophy only, there was statistically significant widespread epilepiiform discharges on the mesial and lateral temporal area in patients with hippocampal and lateral temporal atrophy( p0.3).

The MRI Findings in Complex Partial Seizure Disorders

This study was performed to evaluate, in complex partial seizure, the incidence and nature of MRI abnormality and the correlation between MRI and interictal nasopharyngeal EEG findings. MRI findings were also correlated with age of seizure onset, duration and clinical response to anticonvulsants. For this study, high field MR imaging and interictal nasopharyngeal EEG were performed in 122 patients with complex partial seizures. The patients were distributed in all age groups(mean age:26 years) and include 64 males and 58 females. The average duration of seizure disorder was 10.8 years. Abnormal MRI findings were discovered in 74 of 122 patients, in which mesial temporal sclerosis was most common(58.1%). And other abnormalities were neuroglial tumor(l3.5%), infectious lesion (10.8%), vascular lesion (6.8%), hemiatrophy (4.19@), cystic mass (2.7%) and others (4.1%). There were only slight difference in seizure duration(p0.1).

Clinical and Neuroradiologic Evaluations of Neuronal Migration Disorders

Neuronal migration disorder is a rare group of congenital malfomations of the brain caused by insults to migrating neuroblasts during the six to fifteen gestational weeks. We identified 36 neuronal migration disorders on CTs in two patients and on MRIs in 34 patients and analyzed their characteristic radiologic, clinical, and EEG findings. These 36 patients with neuronal migration disorders consisted of 18 with schizencephaly, eight with pachygyria, five with heterotopias, three with lissencephaly, and two with polymicrogyria. Patient ranged in age from 6 months to 37 years old and mean age was 18.2 years old. Associated cerebral anomalies included ventricular dilatation in 13 patients, agenesis of septum pallucidum and hypoplasia of corpus callosum in nine patients. Lissencephaly was associated with other cerebral anomalies most frequently and all of them had ventricular dilatation and hypoplasia of corpus callosum. Only one patient with pachygyria had ventricular dilatation. Clinically, these patients presented with seizures in 91.7%, speech impairment in 33.3%, abnormal motor function in 30.5%, developmental delay in 27.8%, mental retardation in 25%. Patients with large or medium size of neuronal migration disorders had significantly more severe developmental delay(p=0.001), mental retardation (p=0.004) and speech impairment (p=0.01) than those with small size. Abnormal motor dysfunctions were not significantly associated with lesion size statistically. Seizures did not correlate with lesion size.

Two Cases of Suspected Typhoid Encephalitis Treated with High Dose Dexamethasone

Typhoid fever is one of the important contagious diseases. Typhoid fever of severe category is defined as the condition in which a febrile patient shows shocks or an altered state of consciousness ranging from delirium to coma. High dose dexarnethasone therapy is considered very effective in these life-threatening conditions. We report two severe hrphoid fever cases, which were treated with high dose dexarnethasone (dexamethasone 3mg/kg IV and lmg/kg q6hrs for 48 hrs) with a great success and we believe that one of the two cases is the first Korean case in the literature who had cerebellar ataxia and cortical tremor as a manifestation of the CNS involvement of typhoid fever.

Hemispheric laterality of temporal lobe epilepsy in relation to anxiety and depression / 신경정신의학

No abstract available.

Schizencephaly: An Analysis of Clinical and Neuroradiologic Findings

Schizencephaly is a congenital brain anomaly, a disorder of cell migration characterized by gray matter-lined clefts, which is caused by faulty migration of the subependymal neuroblasts. The cleft may be closed (Type I) or open by a cavity communicating with the lateral ventricle (Type II). We identified the characteristic features of schizencephaly on CTs in one patient and on MRIs in 15 patients. Age at detection ranged from 16 months to 37 years. Three patients had bilateral clefts and 13 patients had unilateral cleft. Twelve patients had closed lip cleft whlle four patients had open lip cleft. Small sized unilateral closed cleft, the most comrnon type, was found in ten patients. Three patients had multiple clefts. Clinically these patients presented with seizures in 15 patients, motor disturbance in six patients, speech impairment in five patients, developemental delay in four patients, and mental retardation in three patients. The presence and severity of these symptoms correlated with the size and location of the lesions. Associated cerebral anomalies included ventricular dilatation in seen patients, agenesis of septum pallucidum in seven patients, other neuronal migration disorder in four patients, agenesis or dysgenesis of corpus callosum in three patients, septo-optic dysplasia in two patients, abnormal extension of svlvian fissure in one patient, and arteriovenous malformation in one patient.

A Case of Syringomyelia Following Cured Tuberculous Meningitis

Syringomyelia is caused by heterogeneous etiologies. Syringomyelia secondary to arachnoiditis complicated by tuberculous meningitis is rarely reported entity, which has not been reported in Korea. We present a patient who had recovered from tuberculous meningitis and developed some years later a progessive disorder of the spinal cord clinically resembling syringomyelia, which was subsequently confirmed by MRI. The patient was treated with syringo-subarachnoid shunt.

Cerebral Arterial Embolism Treated by Intra-Arterial Infusion of Urokinase Which Was Occurred during Percutaneous Balloon Mitral Valvuloplasty

Cerebral arterial embolism which resulted in total occlusion of right proximal middle cerebral artery was occured in 33 years old female patients with severe mitral stenosis and atrial fibrillation during percutaneous mitral balloon valvuloplasty(PMV). The occluded artery could be detected by transfemoral carotid angiography and recanalized immediately by intra-arterial infusion of urokinase. There were no cerebral hemorrhage by CT scan after intra-arterial urokinase infusion and the patients discharged without any neurologic sequale 7 days after thrombolysis. We report a case of successful recanalization with intraarterial infusion of urokinase in patient with cerebral arterial embolism occurred during PMV.

Idiopathic Familial Intracerebral Calcification with Pyramidal Tract Degeneration

A l9-year-old boy was admitted because of progressive spastic quadriparesis and bulbar palsy which developed from 3 years ago. The results of all performed laboratory tests were normal, but neuroradiologic tests revealed intracerebral calcification and degeneration of oilateral pyramidal tracts. Idiopathic familial intracerebral calcification was rarely occurred and presented usually with various extrapyramidal and cortical symptoms. Mild pyramidal symptoms were also associated in some reported cases, but none of them showed predominant spasticity as well as suspicious pyramidal tract degeneration on magnetic resonance imaging.

A Study of Sympathetic Skin Response in Non-Insulin Dependent Diabetic Patients

In order to examine the usefulness of the sympathetic skin response(SSR) as an indicator of autonomic dysfunction, we measured the amplitudes and latencies of the SSR in 64 consecutive non-insulin dependent diabetic patients, which were compared with those of 54 normal controls. The SSR on stimulation of median and posterior tibial nerves with EMG electrographer were correlated with nerve conductoin velocity(NCV) findings of median, posterior tibial and sural nerves and with beat-to-beat variation, measured as difference beeen maximum and minimum heart rate during deep breathing. Diabetic patients were also divided into 5 subgroups according to the symptoms of peripheral neuropathy, autonomic neuropathy and NCV findings for further comparisons. The results were as follows: 1. The shapes of the SSR were similar in the hand and the foot, but the amplitude was consistently greater in the hand than in the foot(p<0.001). The latency was shorter in the hand than in the foot(P<0.001). 2. The latencies of the foot and hand SSR in the diabetic patients were not significantly different from the normal controls. On the other hands, the amnplitude of the hand and foot SSR was significantly reduced compared to the control(p<0.001). 3. Of the patients with absent foot SSR, 20(59%) had two or more symptoms of autonomic involvement, whereas 14(14%) had no autonomic symptoms. These difference were significant(p

Uasbility of Brainstem Auditory Evoked Responses in Preterm and Postterm Neonates

Auditory brainstem potentials were.recorded from scalp electrodes in neonates ranging in gestational age from 33 to 43 weeks, The latencies of the various potential component decreased with maturation, Wave V, evoked by 90-100dB sensation level clicks, changed in latency from 7.18 msec at 33 weeks of gestation to 6.93 msce at 43 weeks of gestation. Central condcuction times( I-V interpeak latency) in the auditory pathway also decreased with maturation from 5.65 msec at 3,3 weeks to 5,35 msec at 43 weeks, Several kinds of patholgic condition including hyperbilirubinemia, bacterial meningitis and respiratory difficulty syndrome were also evaluated with auditory brainstem potentials, the application of all of these techniques could permit an objective definition of normal and will be helpful in abnormal infants.

Comparison between electrophysiologic and morphologic changes in lead induced peripheral neuropathy in rats

Compound nerve action potential (CNAP) of the mixed peripheral nerve is composed of A alpha beta, A delta, and C potentials. All components of CNAPs in the sciatic nerve were recorded by stimulating the tibial nerve of both control and lead-poisoned rats. Marked decrease of nerve conduction velocity and prolonged duration were found in A alpha beta and A delta fibers especially in large myelinated A alpha beta fibers. The amplitude decreased in A alpha beta potential, but the area did not change. In C potential produced by activation of unmyelinated fibers, nerve conduction velocity slightly decreased, but the amplitude and area did not significantly change. Pathologic correlates revealed prominent segmental demyelination with significant decrease of large myelinated fiber densities. Minimal axonal degeneration of unmyelinated fibers was present. We can conclude that electrophysiologic changes in the lead-poisoned rats correlate with pathologic changes in them.

A Case of Herpes Zoster Myelitis

Herpes zoster myelitis is quite rare and it's MRI findings have not been reported. We report a patient who developed a progressive myelopathy after a characteristic herpes zoster skin lesions involving right C3 and C4 dermatomes. The lesions were recognizable in T1-weighted images of MRI as hypointense areas in the central portion of the long segment of the spinal cord, which became more prominent in T2-weighted image as increased signal intensities. In this patient computed tomographic metrizamide myelography (CTMM) showed no detectable intra or extramedullary lesion except a slight bulging of the cervical cord.

A Case of Lambert-Eaton Myasthenic Syndrome

The Lambert-Eaton myasthenic syndrome (LEMS) is a rare disorder of neuromuscular transmission that is frequently associated with malignancy, particulary small cell carcinoma of the lung. To our knowledge, there was no report of LEMS in Korean listeratures and this might be the first case in this country. The patient is a 55 year old man with muscle weakness and fatigability of proximal lower limbs for 2 months. Bronchogenic carcinoma is confirmed radiologically and pathologically. The electrophysiological studies suppert the clinical diagnosis of LEMS with (1) low amplitude of evoked compound muscle potential to a single supramaximal stimulus on nerve, (2) significant decremental response at low rates of stimulation and (3) marked incremental response at high rates of stimulation.

A Clinical Study on Intracerebral Metastatic Tumors

Intracerebral metastases are among the most feared complication of systemic malignant disease and most occur late in the course of the systemic cancer so often are viewed as a terminal maifestation for which diagnosis and treatment are neigher required nor desirable. However, this situation is changing. Improved survival rates with many kinds of cancer have led increasingly to instances where the neurologic complications themselves limit the quality of life, forcing physicians to assume a more active attitude toward the diagnosis and treatment of the neurologic disorder. The authors analysed 125 cases of intracerebral metastatic tumors which are diagnosed with the brain CT scan at Yonsei University, Severance Hospital from January, 1979 to December, 1984 and following results were obtained. 1. Intracerebral metastatic tumors are found in 125 cases(31.2%) of all intracranial neoplasm. 2. The most common primary tumor is lung ca. (50.4%) followed by chorioca. (8%), breast ca. (8%) and GI tract ca. (6.4%) in the order while 16 casese (12.8%) were of unknown primary tumor. 3. Chorioca. (14.9%), melanoma(5.6%), lung ca.(4.2%), kidney ca.(3.3%) and breast ca.(2.3%) exhibit relatively high rate of intracerebral metastasis in the order whereas stomach ca.(0.2%), hepatoma (0.3%) and cervix ca (0.1%), which constitute most common primary maliqnancies in Korea, revealed very low rate of intracerebral metastasis. 4. The youngest patient was 11 years old and the oldest 80. Most intracerebral metastatic tumors, 97%, occur above 30 years of age and peak in the 6th decade (32%). In age below 40, chorioca. Is the most common pimary tumor and the age above 40, lung ca. 5. There is no sexual difference between male and female. In male most conmon primary site is lung (64.6%) followed by GI tract(7.7%)and in female, lung (35%), chorioca. (16.7%) in the order. 6. The most common presenting symptom and sign is headache (58.4%) followed by motor deficit (48.0%), mental deterioration (43.2%) and nausea or vomiting (32.8%). 7. In most of intracerebral metastases the onset is gradual with duration of less than 3 months in 84% whereas in a few patients, 8 cases (6.4%), the onset is apoplectic and the primary sites that revealed apoplectic onset are lun. (3 cases), chorioca. (3 cases), liver (3 cases), unknown (1 cases). 8. Intracerebral metastases are detected simultaneously (50.5%), precociously (3.7%) or after (45.9%) diagnosis of the primary tumor. 9. Average interval between the diagnosis of primary tumor and development of intracerebral metastasis is 9.1 months in total and short in lung, stomach, kidney and liver cancer whereas and long in breast, rectum cancer and sarcoma. 10. In 96 cases who can be reevaluated with brain CT, the findings are as follows; 1) Metastatic lesions are multiple in 67.7% and single in 32.3%. 2) Most intracerebral metastases are located supratentorially (77.%), in 4.2%; infratentorially and in 18.8%; both. 3) Density of lesions are variable from hypodense to hyperdense and intracerebral metastases from chorioca., breast, kidney and nasopharynx ca. Are likely to iso to hyper than hypodense. Calcifications are rare and observed only in 2 cases (2%). 4) Most cases (98.5%) reveal perifocal edema of variable degree while a few patients (11.5%) reveal no edema. 5) Most metastatic lesions demonstrate variable contrast enhancement with nodular type (52.1%), rign type (25.0%), combined (15.6%) and a few patients (7.3%) fail to enhance. 6) Hemorrhages are found in 11 cases (11.5%) and chorioca. (4 cases), hepatoma 3 cases) and lung ca, (3 cases) are the most frequent offenders. 11. Radiation, surgery and chemotherapy seems to improve the prognosis.