A Case of Wilson's Disease Associated with Somatomotor Status Epileticus

Wilson's disease is a genetically transmitted autosomal recessive disorder of copper metabolism which affects the liver. Brain and other organs. The seizure in Wilson's disease is not infrequent but. It's report is very rare in Korea. The frequency of seizure is about 10 times more than that of general population The statistical proportions of types of seizure associated with Wilson's disease do not differ significantly from that seen in the general population. And the most common seizure type is focal motor form. The issue of the mechanisms producing seizure in Wilson's disease has attracted controversy, but the cause of seizure seems to be related with copper itself. Although the seizure is well controlled by anticonvulsants, the prognosis of seizure depends on the treatment of Wilson`s disease. We report a case of Wilson's disease associated with somatomotor status epilepticus.

MR imaging of internuclear ophthalmoplegia due to cerebrovascular diseases / 영남의대학술지

Internuclear ophthalmoplegia is a conjugated gaze disorder characterized by impaired adduction on the side of a lesion involving the medial longitudinal fasciculus with dissociated nystagmus of the other abducting eye. Six patients with INO (who had clinical cerebrovascular diseases) underwent MR imaging and the results were as follows: 1. The MLF lesions were identified by MR imaging in 5 cases 2. The ratio of unilateral INO to bilateral INO was 5:1 3. The nature of lesions was infarction in 4 cases and hemorrhage in 1 case 4. The sites of MLE lesion were in the midbrain in 4 cases and in the pons in 1 case 5. All 5 cases of INO identified by MR imaging had other lesion sites in addition to MLE lesion.

Study on the changes of nerve conduction with wrist fixation in carpal tunnel syndrome / 영남의대학술지

The author studied 20 healthy adults (20 hands) as a control and 30 patients (40 hands) with carpal tunnel syndrome to evaluate the clinical usefulness of measuring nerve conduction velocity after wrist flexion in diagnosis of carpal tunnel syndrome. The median nerve conduction velocity over wrist to finger segment was measured before and after wrist flexion for 1, 2 and 5 minutes, using belly-tendon method for motor nerve distal latency (MNDL) and antidromic method for sensory nerve conduction velocity (SNCV). The results were as follows: 1. In control group, MNDL increased in 1 hand and SNCV decreased in 2 hands after wrist flexion. In patient group, MNDL increased in 2 hands and SNCV decreased in 3 hands after wrist flexion. 2. In both control and patient group, there were no significant changes in mean values of SNCV and MNDL between before and after wrist flexion. 3. Phalen's wrist flexion test was positive in 5 percent of control and 60 percent of patient group. 4. Tinel's sign was present in 10 percent of control and 33 percent of patient group.

A clinical study of acute carbon monoxide intoxication / 영남의대학술지

To obtain the basic data of prognosis of acute carbon monoxide (CO) intoxication, one hundred and sixteen cases of CO intoxication defined by carboxyhemoglobin (COHb) and admitted via emergency room of Yeungnam University Hospital from Oct. '85 to April' 89 have been clinically analyzed and evaluated, including delayed postanoxic encephalopathy (DPE) and the following results were obtained. 1. The ratio of male to female was 1:1.5 and mental state was drowsy mostly (26.2% of 116 cases) 2. The more disturbed the mental state, the more decreased was the arterial pH and PaCO₂, which may be the result of metabolic acidosis. 3. The early laboratory findings in patients of CO intoxication were as follows: leukocytosis-65.5%, increase of hematocrit-23.3%, hyperglycemia-19.8%, increase of GPT-19.8% increase of creatinine-0.9% and glucosuria-12.1%. 4. The early findings of EKG were abnormal in 35.3%: change of rhythm-25.0%, abnormal ST segment 15.5% (change of rhythm and abnormal ST segment-5.2%) but the conduction disorder was not present. 5. The abnormal EEG above mild degree was 93.1%, of which moderate was most frequent (80.2%). 6. The incidence of DPE was 7.8% among all admitted CO patients. DPE cases had long duration of exposure time (8 hours), severe leukocytosis (20,000) and an abnormal EEG (MA).

Endocrine Study on Menstrual Irregularities in Wilson's Disease

We have investigated endocrine functions of 2 patients with Wilson's disease who showed oligomenorrhea or amenorrhea, The serum basal levels of hypothalamic, pituitary, thyroid, adrenal cortical and ovarian hormones were determined. Then stimulation tests were performed with GnRH(50 microgram/m2), TRH(7 microgram/Kg) and insulin(0.l U/Kg) in one intravenous bolus, Levels of LH, FSH, prolactin, GH, TSH, total testosterone, l7 beta-estradiol, free T3, T4, DHEA-S and cortisol were measured by standard radioimmunoassays. The endocrine profiles which represent functions of hypothalamus, pituitary, thyroid and adrenal cortex was all proved to be normal. Serum estradiol and total testosterone levels were 59.0 microgram/ml ( normal ; 60-130 microgram/ml) and 2.9 ng / dl (normal ; 0.l5-1.1 ng/dl) in one case, and 20.5 microgram / ml and 0.69 ng / dl in the other respectiviely. Low estradial and high total testosterone levels seen in these cases suggested ovarian dysfuncton. Interference of ovarian follicular aromatase activity due to passible copper intoxication could explain these findings as the cause of the menstrual irregularity of patients with Wilson's disease.

A Study of Nerve Conduction Velocity of Normal Adults / 영남의대학술지

Nerve conduction studies help delineate the extent and distribution of the neural lesion. The nerve conduction was studied on upper (median, ulnar and radial nerves) and lower (personal, posterior tibial and sural nerves) extremities in 83 healthy subjects 23 to 66 years of age, and normal values were established (Table 1). The mean motor terminal latency (TL): median, 3.6 (±0.6) milliseconds; ulnar, 2.9 (±0.5) milliseconds; radial nerve, 2.3 (±0.4) milliseconds. Mean motor nerve conduction velocity (MNCV) along distal and proximal segments: median, 61.2 (±9.1) (W-E) and 57.8 (±13.2) (E-Ax) meters per second; ulnar, 63.7 (±9.1) (W-E) and 50.6 (±10.0) meters per second. Mean sensory nerve conduction velocity (SNCV): median, 34.7 (±6.7) (F-W), 63.7 (±7.1) (W-E) and 62.8 (±12.3) (E-Ax) meters per second; ulnar, 38.0 (±6.7) (F-W), 63.4 (±7.5) (W-E) and 57.0 (±10.1) (E-Ax) meters per second; radial, 45.3 (±6.8) (F-W) and 64.2 (±11.0) (W-E) meters per second; sural nerve, 43.4 (±6.1) meters per second. The amplitudes of action potential and H-reflex were also standardized. Mean H latency was 28.4 (±3.2) milliseconds. And, the fundamental principles, several factors altering the rate of nerve conduction and clinical application of nerve stimulation techniques were reviewed.

A Case of One and A Half Syndrome / 영남의대학술지

One and a half syndrome is an ipsilateral gaze paresis or palsy combined with and internuclear ophthalmoplegia on contralateral gaze. The lesion site is at the paramedian pontine reticular formation and the adjacent MLF. The common causes are unilateral pontine infarction and multiple sclerosis. We experienced a case of one and a half syndrome which has a suspected small pontine infarct.

A Case of Gelastic Epilepsy, probable Orbito-frontal Origin

Laughter as an epileptic phenomenon is very uncommon. The introduction of the term gelastic epilepsy by Daly and Mulder (1957) may have resulted in less precision in diagnosis. Laughter does not necessarily include Mirth (gelos). Smiling may be volumtary or barely perceptible, whereas the laryngeal and respiratory components of laughter are more likely to be involuntary and are definite. To this time the loction of this epilepsy is said to be related with temporal lobe and hypothalamus. This case which we present with reviewing of the literature has paroxysmally a burst of loud, high-pitched laughter without any emotional expression. It suggests that at the production of this laughter some fasciorespiratory pathways might be involved, and that the start of this epileptic discharge is probably from a lesion of the orbito-frontal area.

Clinical Study of CT-diagnosed Olivopontocerebellar Atrophies / 영남의대학술지

The diagnosis of OPCA could be made clinically with important aid of brain CT scanning, although the definite and conclusive diagnosis only by postmortem pathological determination. We reviewed, clinically and with brain CT examination, 12 cases of patients with OPCA who were admitted to the Yeungnam University Hospital for a recent 5 years. The result were as following: 1. The distribution of age is from 49 to 72, mainly 50 to 60. Man is more frequent than women at the 4.5 times. 2. The interval period from Sx. Onset to diagnosis is 1 year to 6 years. 3. The usual initial Sxs. Were dizziness (58%), ataxia (33%), and other less frequent Sxs. Were weakness of low extremities, dysarthria, headache and urinary incontinence. The clinical manifestations at the initial diagnosis were cerebellar disturbance (100%), dysarthria (83%), and increased deep tendon reflexes (58%). 4. The results of brain CT finding are like this: the width of cerebellar sulci is more than 1mm, other 4 cases more than 2mm. the width of cerebellar pontine cistern of the patient if usually 3 to 4mm, other 2 cases extended to the 5 mm. the A. P and lateral lengths of 4th. ventricle is 4 mm and 4 to 8 mm respectively. 6 cases of whole patients show coincidentally cerebral atrophy.

Etiologic Factors and Prognosis of Delayed Postanoxic Encephalopathy

Etiologic factors and prognosis of delayed postanoxic encephalopathy (DPE) was analyzed in 30 patients admitted in Yeungnam University Medical Center from March, 1984 to April, 1987. Twenty-three among the 30 cases was possible to be followed. The results are as follows. 1. The incidence in over 30 years of age was 77%, and sex ratio was 1:1.7 for male and female. 2. The lucid interval of DPE was from 5 to 191 days (mean=19 days) and interval between 3 to 4 weeks occupied 60%. 3. The longer the duration of exposure to CO and the more comatous of the mental state, the more increased was the incidence of DPE. 4. The hyperbaric O2 threapy had no influence on the incidence of DPE but absolute bed rest on acute CO poisoning ws important to prevent DPE. Therefore, absolute bed rest during lucid interval at least 2 weeks is more important factor to prevent DPE. 5. DPE victims are likely to retire from his social position.

Brain Atrophy and its C.T.Criteria

Since opening of the Yeungnam University Hospital in March 1983 till the end of 1986, total 156 cases of brain atrophy and encephalopathy (control) diagnosed at the neurology department were investigated, adopting C.T. criteria of brain atrophy. Among the 156 only 77 cases had available C.T. films. Of the 77,35 cases were normal (control) and 42 had brain atrophy. The following results are obtained: 1. A new C.T. criteria of brain atrophy and its degree were devised. (Table 8.9) 2. As the age increased, number of brain atrophy cases increased. 3. Degree of EEG abnormalities were not parallel with the degree of brain atrophy. 4. The most frequent EEG abnormalities with or without brain atrophy were moderate abnormalities. 5. The EEG abnormalities were improved with treatment in most of the cases. 6. The group with suspected organic brain lesions had 69% cases of brain atrophy, whereas the group with only headache or dizzines had 57% of no atrophy. 7. There exists a clinical entity of "Cryptogenic Brain Atrophy". (Park) 8. Recent literatures of Brain Atrophy were reviewed.

A Case Report of Conn's Myopathy / 영남의대학술지

Myopathy in primary aldosteronism in relatively rare disease in Korea. A 42-year-old woman with hypokalemic periodic paralysis, proximal muscle weakness and hypertension was found to have myopathy associated with adenoma in the right adrenal gland. She showed marked elevation of muscle enzymes and myopathic pattern in EMG.

A case of Cryptococcal Meningitis / 영남의대학술지

The clinical picture and CSF findings in cryptococcal meningitis may be identical with those of tuberculous meningitis. The differential diagnosis can be made by finding the budding yeast organism in the counting chamber of in stained smear, the detection of cryptococcal antigen in CSF by the latex agglutination test, and by culture of the fungus on Sabouraud agar. We experienced a case of cryptococcal meningitis in the 48 years old woman, which was confirmed by Indian ink preparation and culture.

Antiepileptic Therapy for Latent Epilepsy / 영남의대학술지

The clinical state with EEG pattern similar to interval discharge of epileptics is named as latent epilepsy, which does not necessarily mean that the patient will develop epilepsy later. However, since there is possibility of developing epilepsy on later date, antiepileptic mainly dilantin was tried to control the abnormal EEG. Since January to October 1985, total 580 headache cases with more than moderately abnormal EEG visited the Neurology Clinic. Among them 162 cases with interval seizure pattern (ISP) of epilepsy were selected for the study. The main ISP was 1. diffuse theta and/or delta bursts and 2. spikes. Since the study in only analysis of clinical treatment of 162 cases without previous planning based on financial aid, about 30% of the patients did not return after the 1st EEG examination, in 42% failed to follow the EEG after the treatment and only remaining 28% of the cases were studied. Among 29 patients who were treated with Dilantin 100mg tid po, 16 improved and 13 not. Of the 13, 4 showed partial improvement and partial progression. Case1. In 4 weeks of antiepileptic therapy (AR), spikes disappeared but in 2 months developed bursts. Case2. In 17days of AR, spikes and bursts disappeared but in 3 months bursts reccured. Case3. In 1 week of AR, bursts disappeared but spikes developed. Case4. In 3 months of AR, no change of spikes and bursts and she discontinued the AR. In 6 months she developed grandma seizure. Eighteen cases, treated with other drugs except antiepileptics, all showed improvement. The other drugs were vincaprol, polygammalon, aronamin, ATP and hydergine. The improved cases had spikes more often than theta bursts. In view of the small number of the cases due to dropping most patients out of present study, it is considered meaningless to perform statistical analysis. Further well planned study with more patients is to be expected.

Urokinase Therapy in Cerebral Thrombosis / 영남의대학술지

Since opening of the Yeungnam University Hospital in June 1983 till end of 1984, total 30 cases of cerebral thrombosis were analyzed clinically. Among the 30. 17 cases were given Urokinase from 60,000 to 240,000 units daily for 2-46 days. The interval from the onset to the inititiation of therapy varied from 1 hour to 17 days. Ten cases showed marked recovery and 6 cases moderate improvement. One case, in which only 120,000 units daily were administered, showed stationary course. The interval of the Urokinase untreated 13 cases was from 3 days to 18 months. Six cases were sationary, whereas 7 cases showed mild improvement. Age, sex, past history, associated diseases, and the laboratory findings are shown in the tables. Conclusion is that the earlier the therapy started, the higher the dosage used, the better of the results is obtained.