Neurological Features and Mechanisms of Acute Bilateral Cerebellar Infarction

BACKGROUND: Many studies about unilateral cerebellar infarctions (UCI) have been reported to delineate the clinical findings and stroke mechanisms but have not been studied extensively in acute bilateral cerebellar infarctions (BCI). In order to evaluate the neurological features and mechanisms of BCI, we compared those between BCI and UCI. METHODS: Using diffusion-weighted imaging, we divided 103 patients with acute cerebellar infarctions into two groups: BCI and UCI. Clinical features, outcome and their mechanisms were compared between the groups. RESULTS: Among the 103 patients, 45 patients (44%) had BCI and the remaining 58 patients had UCI. The PICA territory was the most frequently involved site in both groups, and 15 patients were non-territorial infarctions. Clinical symptoms and signs were not different between the groups, however, most patients with decreased mentality had BCI (86%) and also had concomitant lesions outside the cerebellum (72%). Patients with BCI showed poorer prognosis than UCI (modified Rankin score, 1.41 and 2.87 respectively). Other factors included the presence of concomitant lesiona outside the cerebellum, however, mass effect did not affect their prognosis. The main cause of BCI was large artery disease (57%), whereas about half of the patients with UCI (51%) had no demonstrable cause of stroke. CONCLUSIONS: Owing to its poorer outcome and its higher frequency of demonstrable causes of stroke, more intensive work-up, such as vascular study, may be warranted in the patients with acute BCI.

The Clue for Early Diagnosis and Prediction of Intracranial Involvement in Rhinocerebral Mucormycosis

BACKGROUND: Rhinocerebral mucormycosis (RCM) is an uncommon and fatal clinical syndrome resulting from an opportunistic infection caused by a fungus of the order Mucorales in immunocompromized patients. The mortality and morbidity in the patients with intracranial involvement is invariably high, and it was reported that most survivors had early diagnosis and received aggressive treatment. Therefore, we retrospectively reviewed four patients of pathologically confirmed mucormycosis to find out the clues for early diagnosis of RCM and for prediction of fatal intracranial involvement. METHODS: The clinical, radiological features and histo-pathological involvement sites in the patients with intracranial involvement were compared to those without intracranial involvement. RESULTS: All the patients had uncontrolled diabetes and were admitted with orbital involvement. On the precise physical examination, mucosal involvement was found in all patients; three of them in only nasal mucosa and one in oral palatal mucosa. Despite the vigorous antifungal therapy, two patients had intracranial involvement and expired. MRI finding of extensive sphenoid sinusitis adjacent cavernous sinus preceded the intracranial involvement of RCM, which was not found in the patients without intracranial involvement. CONCLUSIONS: Our findings suggest that early observation of oral or nasal mucosal changes might be an important clinical clue for differentiation of RCM from other causes of rhino-oculo-cerebral symptoms in uncontrolled diabetic patients, and that extensive sphenoid sinusitis might be an important radiological feature for predicting the fatal intracranial involvement of RCM.

Diagnosis and Treatment of Headache / 대한내과학회지

No abstract available.

Syndromic Diagnosis at the Epilepsy Clinic: Role of MRI / 대한간질학회지

PURPOSE: To evaluate the application of MRI to the ILAE classification of epilepsies and epileptic syndromes in the setting of epilepsy clinic. METHODS: We reviewed epilepsy registry forms, EEG, and MRI of 300 patients who were consecutively registered to the Yonsei Epilepsy Clinic. The algorhithm of syndromic classification consisted of 3 steps ; 1) clinical diagnosis based on the clinical informations described in the registry form, 2) clinical-EEG correlations, and 3) clinical-EEG-MRI correlations. The interictal epileptiform discharges (IEDs) in EEG were divided into focal, multilobar/multifocal, and generalized. MRI-lesions were divided into focal and non-focal (multilobar/multifocal, and diffuse) lesions. The clinical-EEG, EEG-MRI, and MRI-clinical correlations were categorized as concordant, not discordant, and discordant. RESULTS: Among 300 patients evaluated, 249 patients were found to have epilepsies and both EEG and MRI. By clinical analysis, 190 of 249 patients were diagnosed as localization-related epilepsies (LRE), 24 patients were generalized epilepsies (GE), 34 patients were undetermined epilepsies (UDE), and one patient had alcohol related epilepsy. EEG revealed IEDs in 124 patients and altered the clinical diagnosis in 79 patients. MRI lesions were found in 106 patients with focal lesions in 65 patients and non-focal lesions in 41 patients. MRI lesions were found in 47 of 125 patients with negative EEG. Concordance rates of clinical-EEG, EEG-MRI, and MRI-clinical correlations in 54 patients with lobar epilepsies, who had positive EEG and MRI, were 39%, 54%, and 52%, respectively, and discordant rates were 17%, 11% and 7%, respectively. The complete concordance of all 3 correlations was found in only 33% of them. In 20 patients diagnosed as GE by clinical-EEG correlations, MRI lesions were found in only 3 patients and none of them changed the diagnostic categories due to MRI lesions. CONCLUSION: In lobar epilepsies, the sensitivity of MRI was quite comparable with EEG and the clinical-MRI correlation was superior to the clinical-EEG correlations. MRI provided additional and complimentary informations and should be incorporated to the ILAE-classification system as the category of 'lesional epilepsy'.

A case of kawasaki disease in an adult / 천식및알레르기

Kawasaki disease (KD) is an acute vasculitis of undetermined etiology in infancy and early childhood. There is no diagnostic test to confirm this disease and its diagnosis is made on clinical backgrounds. Most patients diagnosed are under 4 years of age. Here, we report a case of KD in an adult presented with clinical features of fever, headache, and skin rash. A 26-year-old male was presented with fever and headache of four days' duration and skin rash of three days' duration. Nausea and vomiting developed and the patient was treated with antibiotics under the impression of aseptic meningitis and became afebrile two days later. Laboratory findings revealed thrombocytosis, atypical lymphocytes, and elevated liver enzymes. Kawasaki disease was diagnosed, and intravenous immunoglobulin and low-dose aspirin (200mg/d) was administered. Echocardiogram did not reveal any coronary artery changes and the skin lesions disappeared. The patient was discharged after near normalization of the liver enzymes and is now being followed at our clinic.

Effect of Steroid on Brain Tumors and Surround Edemas: Observa tion with Regional Cere b ral Blood Volume (rCBV) Maps of Perfusion MRI

PURPOSE: To observe the hemodynamic change in brain tumors and peritumoral edemas after steroid treat-ment, and then nvestigate the clinical usefulness of perfusion MRI. MATERIALS AND METHODS: We acquired conventional and perfusion MR images in 15 patients with various in-tracranial tumors (4 glioblastoma multiformes, 4 meningiomas, 3 metastatic tumors, 1 anaplastic ependymo-ma, 1 anaplastic astrocytoma, 1 hemangioblastoma, and 1 pilocytic astrocytoma). For perfusion MR imaging, a 1.5T unit employing the gradient-echo EPI technique was used, and further perfusion MR images were ob-tained 2-10 days after intravenous steroid therapy. After processing of the raw data, regional cerebral blood volume (rCBV) maps were reconstructed. The maps were visually evaluated by comparing relative perfusion in brain tumors and peritumoral edemas with that in contralateral white matter. Objective evaluations were performed by comparing the perfusion ratios of brain tumors and peritumoral edemas. RESULTS: Visual evaluations of rCBV maps, showed that in most brain tumors (67%, 10/15), perfusion was high before steroid treatment and showed in (80%, 12/15) decreased afferwards. Objective evaluation, showed that in all brain tumors, perfusion decreased. Visual evaluation of perfusion change in peritumoral edemas revealed change in only one case, but objective evaluation indicated that perfusion decreased signifi-cantly in all seven cases. CONCLUSIONS: rCBV maps acquired by perfusion MR imaging can provide hemodynamic information about brain tumors and peritumoral edemas. Such maps could prove helpful in the preoperative planning of brain tumor surgery and the monitoring of steroid effects during conservative treatment.

Effect of Steroid on Brain Tumors and Surround Edemas: Observa tion with Regional Cere b ral Blood Volume (rCBV) Maps of Perfusion MRI

PURPOSE: To observe the hemodynamic change in brain tumors and peritumoral edemas after steroid treat-ment, and then nvestigate the clinical usefulness of perfusion MRI. MATERIALS AND METHODS: We acquired conventional and perfusion MR images in 15 patients with various in-tracranial tumors (4 glioblastoma multiformes, 4 meningiomas, 3 metastatic tumors, 1 anaplastic ependymo-ma, 1 anaplastic astrocytoma, 1 hemangioblastoma, and 1 pilocytic astrocytoma). For perfusion MR imaging, a 1.5T unit employing the gradient-echo EPI technique was used, and further perfusion MR images were ob-tained 2-10 days after intravenous steroid therapy. After processing of the raw data, regional cerebral blood volume (rCBV) maps were reconstructed. The maps were visually evaluated by comparing relative perfusion in brain tumors and peritumoral edemas with that in contralateral white matter. Objective evaluations were performed by comparing the perfusion ratios of brain tumors and peritumoral edemas. RESULTS: Visual evaluations of rCBV maps, showed that in most brain tumors (67%, 10/15), perfusion was high before steroid treatment and showed in (80%, 12/15) decreased afferwards. Objective evaluation, showed that in all brain tumors, perfusion decreased. Visual evaluation of perfusion change in peritumoral edemas revealed change in only one case, but objective evaluation indicated that perfusion decreased signifi-cantly in all seven cases. CONCLUSIONS: rCBV maps acquired by perfusion MR imaging can provide hemodynamic information about brain tumors and peritumoral edemas. Such maps could prove helpful in the preoperative planning of brain tumor surgery and the monitoring of steroid effects during conservative treatment.

Factors Affecting Prognosis of Temporal Lobe Epilepsy

BACKGROUND: Although the poor short-term outcome of antiepileptic drug (AED) treatment may represent long-term AED refractoriness in general, the same hypothesis in temporal lobe epilepsy (TLE) has yet to come up as an important subject to be investigated. We examined to decide early drug intractability and aid treatment plan. METHODS: From a prospective patient cohort of Yonsei Epilepsy Clinic, 121 newly-referred TLE patients were selected to participate in this study. First, we divided the patients into two groups according to their second one-year remission at two year of AED treatment. One group was the remission group (RG) and the other was the non-remission group (NRG). Second, we evaluated clinical characteristics influencing remission between the two groups. RESULTS: Nineteen patients (15.7%) were RG whereas 102 (84.3%) were NRG. The six month remission and terminal remission at the first year of AED treatment and the first one year remission rate in patients with RG was significantly higher than those with NRG (P<0.05). In patients with NRG, the number of complex partial seizures and secondary generalized seizures, the annual frequency of secondary generalized seizures prior to AED treatment, polypharmacy, new drug use, and drug side effects were significantly higher (p<0.05) than RG. CONCLUSIONS: The earlier seizure outcome may influence the later prognosis of AED treatment in non-lesional TLE. The prognosis during the second year of AED treatment is influenced by drug treatment and the number of generalized seizures.

Cognitive Impairment and Peripheral Neuropathy by Mixed Organic Solvents in Spray Painters Working in a Shipbuilding Industry

BACKGROUND: Exposure to organic solvents becomes a common problem to workers of heavy industries in Korea. A number of volatile organic solvents which are frequently used in painting can cause various derangements of the nervous system, especially cognitive impairments and peripheral neuropathy. METHODS: This study was carried out on 190 workers as a control group who had never been exposed to organic solvents and on 674 spray painters with long-term exposure to organic solvent mixtures. The major components of organic solvent mixtures were determined. All subjects underwent neurological examination as well as routine physical check-up after completing questionnaires on general, musculoskeletal, neuropsychiatric and neurological systems. Subjects with abnormal findings on neurological examination related with the exposure of organic solvent mixtures took further neuropsychological and neurophysiological tests. RESULTS: The prevalence rates of cognitive impairments and of peripheral neuropathy in the exposed group were significantly higher than the control group (9.5% vs 2.1% and 2.1% vs 0%, respectively). High exposure group (more than 2.64 of cumulative exposure index, CEI) showed also higher prevalence of cognitive impairments and of peripheral neuropathy than low exposure group (cognitive impairments; 12.3% vs 6.4%, peripheral neuropathy; 2.3% vs 1.8%). Most common cognitive impairments were attentional deficit, and abnormal sense on the extremities or face was most common symptom of the peripheral nervous system. CONCLUSION: This study is the first large-scale, case-control study extensively evaluating cognitive impairments and peripheral neuropathy related with volatile organic solvent mixtures in spray painters working in a shipbuilding industry in Korea. The results show that the prevalence rates of cognitive impairments and of peripheral neuropathy are relatively low, but clearly related with the exposure extent of organic solvents.

Morphological Alterations of Hippocampus in Temporal Lobe Epilepsy: Cell Loss, Synaptic Reorganization, Cell Birth / 대한간질학회지

No abstract available.

Relationship between Pachymeningeal Enhancement on Brain MRI and CSF leakage on Radioisotope Cisternography in Patients with Orthostatic Headache

BACKGROUND: In orthostatic headache (OH) associated with low cerebrospinal fluid (CSF) pressure, loss of CSF vol-ume reflected by pachymeningeal enhancement (PCE) on brain magnetic resonance image (MRI) has been suggested as a pathogenenesis according to Monro-Kellie rule. We attempted to test the following hypotheses; 1) OH is caused by loss of CSF volume, 2) CSF volume loss in OH is caused by hyperabsorption of CSF or 3) by decreased production of CSF. METHODS: Nineteen patients with OH were recruited. Lumbar puncture, brain MRI and radioisotope cisternogra-phy (RIC) were performed in all of them. We evaluated duration of headache from onset to first evaluation, presence of PCE on MRI and CSF leakage (CSFL) on RIC. Firstly, we compared duration of headache between patients with and without PCE. Secondly, between those with and without PCE, we analyzed presence of CSF fistula and demonstration of CSFL on RIC. RESULTS: Mean duration (16.1 +/-19.6) of headache in 13 patients with PCE (66.7%) was significantly longer than in those without PCE (P=0.036). Among 19 patients, CSF fistula was detected in 13 patients (72%) and CSFL in 16 patients (88.9%). There was no significant difference in CSF fistula presence (P=0.114) and in demonstra-tion of CSFL between those with and without PCE. In 16 patients, delayed appearance of radioisotope along cerebral interhemispheric and sylvian regions was shown on RIC. CONCLUSIONS: Pain in OH may be caused by CSF volume loss, however, whether CSF volume loss is caused by CSF hyperabsorption or decreased production remains to be clarified.

Relationship of Longitudinal Extent of Hippocampal Sclerosis to Clinical Characteristics, Hippocampal Neuronal Cell Loss and Dynorphin-Immunoreactivity Patterns in Intractable Temporal Lobe Epilepsy with Hippocampal Sclerosis / 대한간질학회지

PURPOSE: In mesial temporal lobe epilepsy (TLE). Hippocampl sclerosis (HS) is a pathologic substrate and characterized by significant neuronal loss and band-like synaptic reorganization in dentate inner molecular layer (DGIML) og sclerotic hippocampus with either Timm`s staining or Dynorphin (Dyn)-immunohistochemical staining methods. Hippocampus has neuronal synaptic circuitries of both intralamellar and translamellar patterns, from which we may hypothesize that longitudinal extent of HS represents variable pathophysiologic implications of neuronal injury, ictogenesis and epileptogenesis in mesial TLE. We tested the hypothesis. METHODS: Eleven mesial TLE patients with HS on MRI were recruited from epilepsy surgery registry. Resected hippocampal slices were stained with Dyn immunohistochemical method. We classified them into cases with partial HS and thoes with extensive HS according to longitudinal HS extent,. Between the two groups, clinical characteristics of seizures or epilepaies, Hippocampal neuronal density and neuronal loss. and Dynimmunoreactivity (IR) patterns were compared and analyzed. Dyn-IR pattern was classified as presence or absence of DGIML band and of CA3-IR. RESULTS: Nine cases showed extensive HS whereas two were classified as partial HS. There appeared no significant differences in clinical characteristics, neuronal density, neuronal loss and Dyn-IR patterns between those with extensive and partial HS. CONCLUSION: In this study, we could not prove the hypothesis that difference in HS extend on MRI may represent distinctive variabliity in severity of hippocampal neuronal injury and in ictiogenetic or epileptogenetic pathophysiology.

Difference and Correlation between Seizure Numbers during the First Year and the Second Year of Antiepileptic Drug Treatnent in Temporal Lobe Epilepsy with Mesial Temporal Sclerosis / 대한간질학회지

PURPOSE: Determination of medical intractability is primarily essential for planning a surgical treatment of epolepsy. Questions regarding intractability include the optimum duration of adequate antiepileptic drug (AED) treatment and the tolerable seizure frequency. Unfortunately, thereis no established guideline for determination of medical intractability in terms of the duration or the tolerability of AED resistance. In temporal lobe epilepsy (TLE) with mesial temporal sclerosis (MTS), a Well-Known surgically remediable epileptic syndrome, the inverstigation of relationship between short-term and long-term AED response may help us finding a way to solve the above questions. METHODS: From 2 epilepsy centers, 86 patients with TLE demonstrating MTS on MRI were evaluated. All of them were on AED treatment for at least 2 years. We investigated seizure free rate for 1st 1 year, for next 1 year and that for two years. We compared seizure number of 1st 1 year with that of 2nd 1 year and analyzed corrdlation coefficiency between seizure numbers of the two periods. Paired-t-test and Pearson correlation test were performed for statistical analysis. RESULTS: Two year terminal remission rate was 3.5%. Remission rate for the 1st 1 year was 4.7% Whereas in the 2nd 1 year it rose to 16.3%. Mean seizure number of 1st one year was 34.9 and that of next one year was 27.0. Mean paired differences between seizure numbers of the two periods was 7.95(95% dcnfidence interval:1.82-14.09) Which was statistically significant(p=0.012). Pearson correlation coefficiendy was 0.815 between seizure numbers fo the two periods. CONCLUSION: In TLE with MTS, seizure free outcome was very poor. However, seizurw-free rate rose and seizure number declined as AED treatment duration was prolonged for 2 years. Weizure munber of 1st year may predict that of 2nd year. To establish an appropriate index time for considering medical intractability, further studies on longer-term outcone by AED treatment duration was porlonged for 2 years. Seizure munber of 1st year further studies on longer-term outcome by AED treatment should be recommended in addition to studies on adequate tolerability of AED resistance.

Basic Principles of EEG Interpretation in Epilepsy Patient / 대한간질학회지

No abstract available.

A Case of Sneddon's Syndrome / 대한피부과학회지

Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions with unknown etiopathogenesis. We report a 16-year-old male patient who had been suffered from livedo reticularis for 10 years and multiple cerebral infarction for 2 years. Although the histologic finding of the skin did not show vasculitis and vascular occlusions, the patient revealed livedo reticularis and central nervous system symptoms. These findings were compatible with Sneddon's syndrome.

Radiologic Assessment of Spinal CSF Leakage in Spontaneous Intracranial Hypotension

PURPOSE: To assess the usefulness of imaging modalities in the detection of spinal CSF leakage in spontaneous intracranial hypotension. MATERIALS AND METHODS: Fifteen patients who complained of postural headache without any preceding cause showed typical brain MR findings of intracranial hypotension, including radiologically confirmed CSF leakage. All fifteen underwent brain MRI and radionuclide cisternography. CT myelography was performed in eight patients and spinal MRI in six. Medical records, imaging findings and the incidence of spinal CSF leakage during each modality were retrospectively reviewed. RESULTS: CSF leakage was most common at the cervicothoracic junction, where in seven of 15 cases it was seen on radionuclide cisternography as increased focal paraspinal activity. Leakage was noted at the mid-thoracic level in three patients, at the upper thoracic level in two, and at the cervical and lumbar levels in the remaining two. In two patients multiple CSF leaks were noted, and in all, early radioactive accumulation in the bladder was visualized. CT myelography revealed extrathecal and paraspinal contrast leakage in three of eight patients, and among those who underwent spinal MRI, dural enhancement was observed at the site of CSF leakage in all six, abnormal CSF signal in the neural foramen in one, and epidural CSF collection in one. CONCLUSION: Radionuclide cisternography is a useful method for the detection of CSF leakage in spontaneous intracranial hypotension. CT myelography and spinal MRI help determine the precise location of leakage.

Clinical Characteristics of Ischemic Stroke after Octogenarian Age: A Hospital-based Study

BACKGROUND: Although the incidence of stroke does not decrease after 80 years of age. The significance of ischemic stroke (IS) after octogenarian age has not attracted the attention of neurologists. As a first step to investigate the significance of IS during that period, we compared the clinical characteristics of IS between OIS (octogenarian ischemic stroke group, ??80 years of age) and NOIS (non-octogenarian ischemic stroke group, 65-79 years of age). METHOD: Forty-nine OIS patients and 141 NOIS patients were recruited. Clinical characteristics including risk factors, IS subtype, Canadian Neurological Scale (CNS) score, treatment modality and short-term prognosis were evaluated and described. RESULTS: ypertension (65.3%) was the most common risk factor followed by smoking (28.6%) and previous stroke history (28.6%) in OIS. There was no significant difference in proportion of each IS risk factors between he two groups. OIS was more associated with subtypes of mixed etiology and cardiogenic embolism than NOIS (P.05). IS was more associated with poor outcome at discharge (52.2%) than NOIS (18.2%)(P<.05). CONCLUSIONS: Ischemic stroke after octogenarian age is characterized by different etiopathogenesis and poor short-term outcome compared to IS under that age. OIS is more frequently caused by cardiogenic embolism or combined underlying etiology, however, the relationship between differences in etiopatho-genesis and poor short-term outcome remains to be clarified.

Reappraisal and Practical Application of International League Against Epilepsy ( ILAE 1989 ) Classification of Localization-Related Epilepsies in Adult

BACKGROUND: Since 1985 when ILAE proposed its first classification system of epilepsy, many studies have reported the practical applicability of the system. However, its limitations have been elucidated. In order to find out the applicability and limitations of the ILAE classification system and the role that diagnostic parameters (semiology, EEG and MRI) take in the anatomical localization of localization-related epilepsies (LREs), we investigated the clinical data of adult patients with LRE in step-wise way. METHOD: We recruited 173 patients with newly-referred/diagnosed LRE from our departmental data registry. Idiopathic epilepsies were excluded. We evaluated the anatomical localization rate(LR) according to each diagnostic parameter, the concordant localization rate(CLR) between two parameters and between three parameters. LR in total patients by any one of three diagnostic parameters was also evaluated. MRI abnormalities were evaluated in those patients showing concordant localization between semiology and EEG. RESULTS: The highest anatomical LR(67.1%) was reported in the semiological parameter. CLR between semiology and EEG was 28.9%. CLR between three parameters was 16.2%. MRI abnormalities were seen in 60% of patients with concordant localization between semiology and EEG. Fifty six percent of electroclinically concordant patients showed concordant localization with an MRI and 79% of them were concordantly localized in the temporal lobe. The LR in total patients was 71.7%. In each of the evaluation steps, the temporal lobe LR was the highest. CONCLUSIONS: Total lobar LR by any one diagnostic parameter in all the patients was high according to the ILAE diagnostic criteria. Semiology was the best localizing parameter, however, combined evaluation with either EEG or MRI reduced the localizability. Even though the MRI study showed a significant discordance rate in patients with electroclinical localizations, it could identify the underlying etiology in a major proportion of the patients. This study showed the importance of an imaging study in the lobar localization of LREs combined with an electroclinical localization by the ILAE classification system.

6 cases of postinfectious opsoclonus in adult

Opsoclonus is the involuntary, repetitive, rapid conjugate ocular movements occurring in all directions that are irregular in amplitude and frequency. The cause of opsoclonus are idiopathic, paraneoplastic or postinfectious. Opsoclonus of the postinfectious origin often accompanies other abnormal movements such as tremor, myoclonus and ataxia, and shows a relatively benign course with good response to steroid. Six patients who suffered from myalgia, chilling, fever, cough and generalized weakness fior one to two weeks prior to the admission, experienced visual disturbance, gait unsteadiness and clumsiness of the upper extremities,, Prominent opsoclonus, tremulous movements of trunk and limbs, and intermittent myoclonus were observed. These symptoms markedly improved several days following the use of ACTH or dexametasone.

6 cases of postinfectious opsoclonus in adult

Opsoclonus is the involuntary, repetitive, rapid conjugate ocular movements occurring in all directions that are irregular in amplitude and frequency. The cause of opsoclonus are idiopathic, paraneoplastic or postinfectious. Opsoclonus of the postinfectious origin often accompanies other abnormal movements such as tremor, myoclonus and ataxia, and shows a relatively benign course with good response to steroid. Six patients who suffered from myalgia, chilling, fever, cough and generalized weakness fior one to two weeks prior to the admission, experienced visual disturbance, gait unsteadiness and clumsiness of the upper extremities,, Prominent opsoclonus, tremulous movements of trunk and limbs, and intermittent myoclonus were observed. These symptoms markedly improved several days following the use of ACTH or dexametasone.