Clinical Competence and Organizational Socialization according to Communication Style of Preceptors as Perceived by New Nurses / 기본간호학회지

PURPOSE: The purpose of this study was to examine clinical competence and organizational socialization according to communication style of preceptors as perceived by new nurses. METHODS: From May 1 to July 31, 2014, data were collected from 180 new graduated nurses who had been working for 3~12 months in hospitals in Busan and one in Gyungnam. Data were analyzed using descriptive statistics, t-test, ANOVA, and Pearson correlation coefficients. RESULTS: Preceptors's communication style perceived by the new nurses included supportive type, reflective type, emotive type, and directive type in order of precedence. The average score for clinical competence was 2.82+/-0.31, and the average score for organizational socialization was 3.05+/-0.40. There were significant differences in nurses' clinical competence(F=9.087, p<.001) and organizational socialization (F=30.129, p<.001) according to preceptor's communication style. CONCLUSION: Results indicate that supportive and emotive communication styles of preceptors were important factors in the success of the preceptees' clinical competence and organizational socialization.

Evaluation of the Automated QWALYS-3 System for ABO and RhD Grouping and Unexpected Antibody Screening / 대한수혈학회지

BACKGROUND: One hundred years after the discovery of the ABO blood group system by Landsteiner, agglutination is still the most widely used method for determining the blood grouping and for antibody detection. Yet the demand for suitable automated systems is obvious in the field of transfusion. QWALYS-3 (DIAGAST, Loos Cedex, France) is a fully automated system for determining the ABO and RhD grouping and for antibody screening. We evaluated the ABO and RhD grouping and antibody screening test of the QWALYS-3. METHODS: The ABO and RhD grouping and the antibody screening test were performed by the QWALYS-3 system and the results were compared to the manual methods for the ABO and RhD grouping and to the micro column agglutination method (DiaMed-ID system) for antibody screening. RESULTS: For 574 of 574 clinical samples, there was complete concordance between the QWALYS-3 and the manual methods for determining the ABO and RhD grouping. The concordance rate between the QWALYS-3 and the micro column agglutination method for antibody screening in 210 clinical samples was 97.1%. CONCLUSION: The QWALYS-3 system had a good concordance rate compared to the manual method and the micro column agglutination method. The QWALYS-3 system was robust and it gave accurate results during this evaluation. The QWALYS-3 system will contribute to achieving full automation of all the necessary blood bank tests.

Premature Reversal Caloric Nystagmus in a Patient withMedulloblastoma, Originated from the 4th Ventricle

Some individuals may demonstrate a secondary phase nystagmus (SPN) following the caloric irrigation. It has been stated that if a SPN begins prior to 140 seconds after the onset of caloric stimulation and if the magnitude of the slow phase velocity is greater than 6 degrees/sec, then a premature reversal caloric nystagmus (PRCN) is said to exit. Thus far, there have been no reports describing PRCN in Korea. We described a typical PRCN in a patient with medulloblastoma within the 4th ventricle. The patient had a gaze-evoked horizontal and upbeating nystagmus. However, there was no indication of spontaneous nystagmus. A monothermal caloric test was administered. The initial left beating primary phase nystagmus was subsided at 60 seconds after right cold water stimulation and at 110 seconds, a right beating SPN with 14 degrees/sec of slow phase velocity was appeared and was continuous for 240 seconds. Although the precise mechanism of PRCN is unknown, vestibular nuclei damage may be related.

Vestibular Function Test of Vestibular Neuritis in Acute and Compensated Stage

BACKGROUND: Vestibular neuritis (VN) is a common peripheral vestibulopathy. VN is most likely a partial rather than a complete vestibular paralysis, It has a natural history of gradual recovery within 1-6weeks. The investigation of vestibulo-ocular reflex (VOR) change in acute and compensated VN are relatively few in Korea. We performed the vestibular function test including electronystagmography (ENG) and rotary chair test (RCT) in the patients with acute and compensated VN, and evaluated the efficacy of ENG and RCT to know the degree of compensation. METHODS: Tweenty-four patients with acute VN, 14 patients who had cllinically compensated VN during follow-up period and 30 normal controls were studied. Mean intervals from symptom onset to test were 3.6 days (acute) and 102.5 days (com-pensated). RESULTS: Eight patients had asymmetrically impaired pursuit, and ten patients had asymmetrcally impaired OKN during acute stage. The degree of side differences in pursuit and OKN gain was correlated with intensity of spon-taneous nystagmus. In the acute stage, the gain of the VOR was reduced at low frequency (0.01-0.16Hz), but it was normal at high frequency (0.32Hz). Prolonged phase lead and gain asymmetries were present at all range of frequency. In the compensated stage, the gain, phase and symmetry of the VOR at all range of frequency were not different from those of controls, except for prolonged phase lead and asymmetry at 0.01 Hz. The rate of the patients with unilateral canal paresis was 100% at acute stage and 50% at compensated stage in mono-thermal cold caloric stimulation. CONCLUSIONS: These results suggested that pursuit and OKN abnormalities may be found in acute peripheral vestibu-lopathy, in which coarse spontaneous nystagmus may contribute to the development of these abnormality. Absence of caloric response does not indicate an complete absence of vestibular function and RCT is a useful method in evaluation of VOR status in patients with acute and compensated VN.

Prognosis of Temporal Lobectomy for Temporal Lobe Epilepsy Patients with Mental Retardation

BACKGROUND: For decades, patients with epilepsy and mental retardation (MR) have been considered as a relative contraindication for focal resective epilepsy surgery. However, considerable debate exists concerning whether the presence of MR is a contraindication for epilepsy surgery or not. We examined the postsurgical seizure outcome of temporal lobe epilepsy (TLE) according to preoperative intelligence scores and the relationship between preoperative intelligence and postsurgical seizure outcomes of TLE. METHODS: We investigated 131 patients with TLE divided into two groups according to standard IQ ranges and seizure outcomes were compared by Engel's classification. RESULTS: The percentage of seizure free patients after surgery was 66.4% in all patients and the percentage in patients with and without MR were 60.7% and 68%, respectively. The age at seizure onset was significantly different between the MR group and non the MR group. However, other factors which have been thought to affect the intelligence of patients with TLE were not significantly different between the two groups. The best postoperative seizure outcome was shown in the borderline group, where the frequency of hippocampal sclerosis and age at seizure onset were significantly higher compared to the other groups. CONCLUSIONS: Although preoperative MR has been regarded as a predictor for a poor prognosis of surgical outcome, our results suggest that MR alone is not a contraindication for epilepsy surgery. Therefore, epilepsy patients with MR who meet other inclusion criteria for epilepsy surgery should not be rejected for epilepsy surgery.

Migraine and Sudden Hearing Loss

On rare occasions, sudden hearing loss may be associated with a migraine. To our knowledge, there have only been two prior reports of sudden hearing loss attributed to migraines. A 40 years old man had suffered from recurrent headaches with hemiparesis, vertigo, and hearing loss for the past three years. At this time, he presented with severe bilateral sensorineural hearing loss of sudden onset, anarthria, right hemiplegia, tinnitus, and vertigo. An audiogram showed a down-sloping sensorineural hearing loss of 77dB by pure tone average on the right side and 72dB on the left. Sixteen days later, audiographic findings improved to 30dB of pure tone average on both sides. Vasospasm of the labyrinthine arteries instigated by migraine might explain these inner ear symptoms. A history of migraine should be sought in patients with sudden hearing loss and when found, anti-migraine medications such as calcium channel block-ers should be considered.

Ischemic Stroke After Acute Myocardial Infarction

BACKGROUND: The relationship between the anterior site of acute myocardial infarction(MI) and occurrence of stroke has become a recent subject of much debate in the relevant literature. The object of this study was to examine the incidence of ischemic stroke during the hospitalization after an acute MI and to identify predictors of MI-related stroke. METHOD: We performed retrospective analysis of 452 patients with acute MI admitted to department of Neurology or Cardiology from January 1990 to August 1997. Patients with a stroke during hospitalization after acute MI were recruited for this study. RESULT: Thirteen cases were recorded. Seventy-seven percent (10/13) of the strokes occurred within 4 days after onset of MI. Multivariate analysis identified the following as independent predictors of stroke : History of hypertension(OR. 2.6: CI, 1.1 to 5.9), previous stroke(OR, 22.3: CI, 5.9 to 84.9) and congestive heart failure (CHF)(OR, 15.4: CI, 2.2-108.6). Transthoracic echocardiography(TTE) was performed in 349/452(77%) during hospitalization. The incidence of left ventricular thrombosis(LVT) in patients with anterior MI who received thrombolytic and anticoagulant therapy was not significantly different from that in patients with anterior MI who didn't(2.6% vs 6.1%, p=0.265). Stroke developed in only one of these patients with LVT. Thrombolytic and anticoagulant therapy were more frequently used in patients without stroke compared with stroke.(p<0.05). CONCLUSION: The incidence of stroke after acute MI is 2.9% and more frequent within the 4 days after MI. History of hypertension, previous stroke and congestive heart failure (CHF) were the factors independently favoring the occurrence of stroke, but there is no relation between the occurrence of stroke and anterior site of MI. Although intravenous thrombolytics followed by full anticoagulation treatment appeared to provide protection against ischemic stroke, it has no effect in the incidence of left ventricular thrombi in this study.

Stroke as a Late Complication in Patients with Cardiac Prosthetic Valves

BACKGROUND: Information on the long-term fate of patients with prosthetic valve is limited. Cerebral embolism is a major cause of late morbidity and mortality in patients with prosthetic valves even though recent prostheses are less thrombogenic and anticoagulants are administered. We investigated the long-term risk of the first ischemic and hemorrhagic stroke and effect of presumed risk factors on the development of stroke in patients with prosthetic valves. METHODS: In a retrospective study, 554 patients who survived the 30-days after cardiac valve replacement between June, 1985 and May, 1995 were included. End points were attacks of ischemic and hemorrhagic stroke. Risk of stroke at end points was calculated according to Kaplan-Meier method. The influence of several clinical variables on these stroke events was analyzed by univariate and mutivariate analyses. RESULTS: Thirty seven of 554 patients had 42 stroke events(34 ischemic stroke, 8 hemorrhagic stroke) during follow-up periods(mean: 52.9months). Six of these patients died from stroke. The cumulative stroke-free probability was 92.4% at 5years and 87.7% at 10years. The cumulative ischemic stroke-free probability was 93.7% at 5years and 89.4% at 10years. No or irregular use of anticoagulants was the only significant independent predictor(RR:4.99, 95%CI:2.01-12.41, p<0.01) of ischemic stroke. The cumulative hemorrhagic stroke-free probability was 98.7% at 5years and 98.1% at 10years. CONCLUSION: Regardless of the type of prostheses, patients with prosthetic valves, notably those without anticoagulants, are at high risk for ischemic stroke.

Interictal scalp EEG in intractable temporal lobe epilepsy

BACKGROUNDS: The localization validity of interictal scalp EEG abnormalities in the patients with temporal lobe epilepsy(TLE) has been a subject of much debate. This study was an attempt to assess the general characteristics and the lateralization value of interictal scalp EEG abnormalities of TLE. We also examined the possible etiologic factors of bitemporal independent epileptiform discharges(BIED). METHODS: We investigated the interictal scalp EEGs of 59 patients. All patients have been seizure free but might have had persistent auras (Engel's classification, class I) after anterior temporal lobectomy(ATL) with minimum follow-up of 1 year. Long term scalp EEG monitoring records were evaluated for interictal EEG abnormalities in all patients. RESULTS: Scalp EEGs from 59 patients exhibited clear epileptiform discharges. Strictly unitemporal epileptiform discharge(UED) was present in 23 patients (39%). It was concordant with the side of seizure origin in 22 patients (95%), and discordant in 1 patient (5%). Thirty six patients (61%) had BIED. Twenty two patients with BIED showed lateralized preponderance which was defined as at least 80% laterality. It was concordant with the side of seizure origin in 21 patients (95%), and discordant in 1 patient (5%), and 14 patients were not lateralized. Bilateral synchronous epileptiform discharges were present in 4 patients (7%), and extratemporal spike in only 1 patient (2%). Localized temporal slow waves were shown in 33 patients (56%). It was concordant with the side of seizure origin in 28 patients, discordant in 1 patient. Four of 33 patients had bilateral temporal slow waves. There was no statistically significant difference in age at seizure onset, duration of epilepsy between the groups with UED and with BIED. Febrile seizures occurred similarly in both groups. CONCLUSIONS: It seems obvious that patients with UED or lateralized interictal temporal spike or sharp waves have a strong likelihood of ictal onset from the ipsilateral temporal region.

Unification of Oriental and Western medicine with study on Oriental and Western medicine / 의사학

In Korea the system of medical management has been bifurcated into two parts since the restoration of nation from Japanese after World War II. One is 'Western Medicine' and the other is 'Oriental Medicine' like Chinese but not like Japanese. The authors attempted to study on the unification of both medicines to prevent the confusion of medicare for people. The major part of medical care is accomplished by Western medicine among people in Korea and China. However, Oriental medicine is still present as well as college of Oriental medicine in small number. There have been long struggles between two parts of medicine because of each assertions for the theories. The ancient medicine has been likely to have its characteristics either in Western or Oriental medicine although in many countries were succeeded by Western medicine except a few Asian countries such as Korea and China. In Japan since the license of Oriental(herb) medicine was ceased about one hundred years ago, the Western medicine has been authorized by law until today and the herb is the secondary medicine as a kind of folk treatment as well as continuous and enormous study for scientification. In only China and Korea this herb medicine has been kept to use as a part of medicine by law though China has developed combined medicine to use both Western and Oriental medicine by one physician since previous prime minister Chou En-Lai around the year 1950 who made a recommendation to the two medical societies, Western and Oriental. This fact has a big sense to establish the unification of two medicines in near future in Korea as well as China. For this accomplishment of medical unification both parts of medicine require sincere and enormous efforts to study the theory and practice of herb medicine even from now on. This unification of two medicines will provide the best medicare in most convenient way to the people in the world creating new world medicine like the third medicine including alternative medicine. The acupuncture is changing to a new method in Western medicine especially applying into anesthesia and pain clinic in even USA and Western countries at this time. In conclusion the unification of the Western and Oriental medicine will provide the most reasonable and practical medicare including alternative medicine to people in the world. Because the Oriental medicine will be used at least separately to supplement the Western medicine by one physician

A case of neurosarcoidosis without systemic involvement

Sarcoidosis is a multisystem disorder of unknown cause. The involvement of the nervous system occurs 5% to 27% of patients with sarcoidosis, and neurosarcoidosis without systemic involvement is rare and difficult to diagnose. We present a case of 58-year-old woman with clinical features of multiple cranial and peripheral polyneuropathy with noncaseating granulomatous inflammation. Extensive testing for occult systemic sarcoidosis was negative. Sural nerve biopsy showed several perineural noncaseous granulomatous inflammation with prominent epithelioid cells. Oral steroid therapy led to some improvement. We report a patient with multiple cranial and peripheral polyneuropathy without systemic involvement, suspected sarcoidosis.

Vestibular dysfunction in patients with idiopathic parkinson's disease

BACKGROUND AND OBJECTIVES: Qualitative oculomotor abnormalities have been reported in parkinsonian patients for many years, but conflicting results have been obtained. This study was performed to evaluate the correlation between the severity of the disease and the abnormalities of the ocular movements in idiopathic parkinson's disease. METHODS: We gave the vestibular function tests in patients with idiopathic parkinson's disease and normal controls. Eye movement recordings were made with automated electronystagmography and rotation test was performed. A total of 46 patients (mean age : 61.2+/-6.7) and 24 controls (mean age : 60.5+/-4.3) were studied. The severity of the disease was divided into two groups by modified Hoehn & Yahr staging ; H-Y stage 1 and 2 as a mild group and stage 3 and 4 as a severe group. RESULTS: Saccadic latency and accuracy, pursuitic gain and velocity, vestibulo-ocular reflex (VOR) suppression by vision were significantly altered in patients, whereas mean velocity of optokinetic nystagmus (OKN) and VOR gain in darkness were normal. Alteration of saccadic latency and accuracy, pursuitic gain and velocity, VOR suppression by vision were profound in the severe group compared with a mild group and controls, but the above parameters did not differ between a mild group and controls. In a hemiparkinson's group, saccadic latency and accuracy, pursuitic gain, OKN mean velocity and gain was not different between the both sides. CONCLUSION: The results indicate that severe Parkinson's disease damages nigrostriatal or other specific pathways which were involved in the regulation of the saccadic, pursuitic and pursuitic-mediated visual fixation system. In a hemiparkinson's group asymmetric damage of dopaminergic innervation which was involved in the regulation of ocular movements was not found.

The expression of the immediate early genes, c-fos, krox-24 and the late response gene, BKNF induced by antiepileptic drugs

The expression of the c-fos and krox-24 (immediate early genes: IEGs) and the BDNF (late response gene) were investigated by convulsants such as kainate (KA, 200 micrometer), N-methyl-D-aspartate (NMDA, 10 mM), glutamate (GLU, 2 mM), and picrotoxin (PTX, 20 micrometer in the rat C6 glioma cells. In addition, the changes of their expression patterns were investigated by the anticonvulsants such as a NMDA antagonist MK-801, phenytoin, phenobarbiw, diazepam, and newer antiepileptic drugs like felbamate and gabapentin. NMDA induced c-fos and krox-24 expromiom were decreased spatially by the anticonvulsants. KA, NMDA, GLU, and PTX-induced BDNF expression were increased by the anticonvulsants. These results imply the molecular basis of the anticonvulsant action mechanism lies in differential and coordinated transcriptional regulation of IEGs.

Changes in frequency of seizure after acute antiepileptic drugs withdrawal

This study was performed to evaluate the effects of acute withdrawal of antiepileptic drugs in epileptic patients during continuous BEG monitoring. One hundred sixty-five withdrawals in 134 patients who were candidates for epileptic surgery were included for this study. Clinical features and frequency of seizure were observed after drug withdrawal with daily monitoring of serum drug level. The phases after withdrawal of antiepileptics were divided into phase of therapeutic drug level, phase of falling drug level, and phase of subtherapeutic or undetectable(zero) drug level. There were significant increase in frequency of seizure and seizure of secondary generalization after acute withdrawal of antiepileptic drugs. Number of seizure during the period of drugs withdrawals was not correlated with onset age of epilepsy, duration of epilepsy, duration of medication, and number of administrated antiepiteptic drugs. The number of frequency of seizure before drug withdrawal was correlated with the number of frequency of withdrawal seizure. The number of seizure frequency after carbamazepine withdrawal was significantly higher during the phase of subtherapeutic or zero drug level, and not during phase of rapid falling antiepileptic drug level.

Knowledge and attitude toward epilepsy in some Taegu-kyungbook residents

The previous studies had shown that our society had stigma about epilepsy. These studies were not enough to establish practical and concrete educational program for the general population. This study was carried out to evaluate the knowledge of and attitude toward epilepsy in general population. The subjects consist of the population of 1722 apparently healthy persons who reside in Taegu city and Kyungpook province. The questionnaire consists of 39 items concerning knowledge of epilepsy and 20 items of attitude toward epilepsy and epileptic patients. Seventy percent of respondents answered that epileptics have grand mal seizure as their unique type of seizure. Sixty-eight percent were not confident of drug therapy for epilepsy, and 53% considered that epilepsy is a genetic disorder. The score of negative attitude toward car driving and marriage in epileptics was high. The score of knowledge of epilepsy and of attitude toward epilepsy and epileptics show a significant correlation with age and level of education. Although the group of public servant, teacher and professor had relatively high score in knowledge of epilepsy, they had much more negative attitude toward driving and marriage of epileptics. The group of nurse-teacher had higher knowledge and much more affirmative attitude. It is suggested that an educational program to epilepsy should have special emphasis on the etiology, type of epilepsy and efficacy of medical treatment in epilepsy.

The clinical study of benign paroxysmal positional vertigo of horizontal semicircular canal origin

BACKGROUND & OBJECTIVES: It was well known that benign paroxysmal positional vertigo(BPPV) was caused by the involvement of the posterior semicircular canal, but there were a few reports about horizontal canal variant of BPPV. We studied the clinical features electronystagmographic (ENG) findings and the possible mechanism of BPPV of the horizontal canal(HBPPV). METHODS: We examined 10 cases of HBPPV with episodic vertigo and intense horizontal geotropic nystagmus provoked by rotation of the head in a supine position. Six patients underwent ENG and we recorded four patients' nystagmus, provoked by rotating head from extreme lateral, mid and nose-up position to extreme contralateral direction in supine position. RESULTS: All observed horizontal nystagmus beated towards the ground on both sides and were more pronounced when head was rotated to pathological side. In rotating to pathological side, nystagmus had mean latency of 2.6+/-1.8 seconds, peak velocity of slow phase of 66,8+/-19.7 degree/second and mean duration of 24.7+/-3.4 seconds. In rotating to healthy side the nystagmus had 3. 7+/- 2.9, 38.0+/-11.5 and 22.5+/-4. Secondary phase nystagmus occurred in 2 patients and fatigue was observed in 4 patients. The slow phase velocity of nystagmus was relative to the distance of head rotation. The duration of attack is 3 to 17 days(6.9+/-5.2days). Liberatory manuever had relatively a good effect. CONCLUSION: HBPPV represents the origin of horizontal semicircular canal and has a good prognosis Above findings support that mechanism of horizontal BPPV is canalolithiasis.

Serum Anti-Acetylcholine Receptor Antibody, Interleukin-2 and Soluble Interleukin-2 Receptor Level in Myasthenia Gvavis

Objective/BACKGROUND: Serum levels of anti-acetylcholine receptor antibody (AChR-Ab), interleukin-2 (IL-2) and soluble interleukin-2 receptor (sIL-2R) may represent markers of disease severity in myasthenia gravis (MG). This study was performed to evaluate the correlations between disease severity and immunological parameters such as serum AChR-Ab, IL-2 and sIL-2R level and between each immunological parameters. METHODS: Serum levels of AChR-Ab, IL-2 and sIL-2R were measured in 30 MG patients and in 22 healthy controls. Results : Serum levels of AChR-Ab and sIL-2R were higher in MG than in healthy controls (p<0,01). The occurrence of IL-2 positive serum samples was 46.7% in MG but none in controls. There were no significant correlations between disease severity and immunological parameters and between each immunological parameters. Conclusions : Serum IL-2 and sIL-2R levels may not represent markers of disease severity in MG, In order to further document the correlation between each parameters, follow-up of individual patients with serial serum samplings may be necessary.

Clinical Study of Benign Childhood Epilepsy with Occipital Paroxysms(BCEOP)

BACKGROUND & OBJECTIVES: Although benign childhood epilepsy with controtemporal spikes (BCECT) has been well characterized and extensively studied, the clinical findings and prognosis of benign childhood epilepsy with occipital paroxysms (BCEOP) have been less well understood. The purpose of this study was to evaluate clinical manifestations, responses to antiepileptic drugs, and longterm. Prognosis of BCEOP. METHODS AND SUBJECTS: We studied 10 BCEOP patients seen between 1985 and 1995 and followed up from 2 to 14 years(7.2+/-2.9). RESULTS: In 10 patients, there were 7 females and 3 males. The age of onset ranges from 4 to 9 year-old(6.4+/-1.5). In the cases whose age of onset was below 6 year-old, the main clinical manifestations were tonic deviation of eye bal1, ictal vomiting and frequent development of status epilepticus in the sleep. In the cases whose age of onset was above 7 year-old, the main clinical manifestations were visual symptoms (blurring or blindness) associated with ictal vomiting/headache and complex visual hallucination evolved to automatism. The typical EEG findings were occipital paroxysms, characterized by repetitive high amplitude spike or sharp and slow wave complexes in the occipital area, mainly when the eyes were closed. Except one patient, the seizures responded well to anticonvulsant therapy and did not occur after the age of 11. CONCLUSION: Although the number of cases was small and the follow-up period was not long enough, our data indicated that BCEOP had a good prognosis and showed diverse clinical manifestations, which might be dependent on the age of onset.

A Case of Non-paralytic Pontine Exotropia in Brain Stem Infarction

Paralytic pontine extropia(PPE) is a exotropia in contralateral eye of lesion side, associated with one-and- a-half syndrome in acute phase of brainstem infarction and after then, often followed by non-paralytic pontine extropia(NPPE) and medial longitudinal fasciculus (MLF) syndrome in each clinical improvement. NPPE is pontine exotropia without lateral gaze palsy to ipsilateral side and is considered to be due to partial impairment of the unilateral paramedian pontine reticular formation(PPRF). We reported a case, 75 yearold man, of brainstem infarction who initially presented NPPE in acute phase, followed by ipsialteral MLF syndrome after 5th days.

Two Cases of Lafora's Disease

Lafora's disease is one of the major causes of progressive myoclonic epilepsy. The main clinical manifestrations are epilepsy, both generalized and focal, severe and progressive myoclonus, progressive dementia and cerebellar sign, then leading to death within 2-10 years. The definite diagnosis depends on the detection of the characteristic PAS positive inclusions, which are present in various tissues including the brain, liver, muscle and skin. We presented two brothers who showed typical clinical features of this disorder, confirmed by skin and muscle biopsy.