Pathological laughing and crying(PLC) is a condition that is characterized by episodic, brief, contextually inappropriate, uncontrollable outbursts of laughing and/or crying. It can be observed in patients with various neurological disorders. PLC often causes distress in interpersonal functioning and activities for patients and their families. PLC can be recognized easily with proper understanding of the condition and its nature. Also it generally shows good response to various pharmacological treatments. This review aims to encourage the diagnosis and treatment of PLC by providing definition and clinical presentation of PLC, analysis of its pathophysiology and various current treatment options.
Crying* ; Diagnosis ; Humans ; Nervous System Diseases

The purpose of this study w-s to predict the respose to the chincap therapy from the initial cephalometric measurements and to obtain the indication of chincap therapy. 40 patients selected for this study were classified into two groups by the occlusal stability after completion of permanent dentition and the iiu4n ovement of facial profile, after chincap therapy. One was g response group which consisted of 25 children and the other was poor response group with 15 patients. Various measurements of the craniofacial structure in the initial lateral cephalogram were calculated and analyzed by t-test and discriminant analysis. The results were, as follows 1. Good response group had more horizontal growth pattern in initial stage of treatment, and the contributing measurements were Bjork sum, anterior-posterior facial height ratio, gonial angle, lower gonial angle and occlusal plane to AB plane angle. 2. The critical points and predictive values of the influential skeletal measurements were calculated. 3. The discriminant function was obtained from three. major influential measurements; Bjork sum, gonial angle and occliml plane to AB plane angle, and this function could discreminate correctly in 85% of this samples.
Child ; Dental Occlusion ; Dentition, Permanent ; Humans

The Pill Questionnaire (PillQ) has been proposed as a simple way to evaluate cognitive deficits and their impact on the daily lives of those with Parkinson’s disease (PD) by asking patients or caregivers about whether patients can independently manage their pills. We used the PillQ to investigate the association of ability to manage medication with cognition and activities of daily living (ADLs) in patients with PD. Patients were divided into two groups based on PillQ scores. The no-impact group was able to take their antiparkinsonian medication independently, and the impact group exhibited problems describing their treatment or taking their drugs independently. A total of 208 participants (93 men) were included. 111 patients (53.4%) were included in the no-impact group, and 97 (46.6%) were included in the impact group. The impact group showed significantly lower cognitive functioning, difficulties with the performance of ADLs, and severe motor dysfunction. PillQ scores were significantly correlated with Mini-Mental State Examination and the Montreal Cognitive Assessment, and Clinical Dementia Rating scores. Management of medication by PD patients is associated with cognitive function, and the PillQ is an easy and useful test for detecting cognitive impairment and its impact on daily life.

Although most of the known pathogenic mutations in the progranulin gene (PGRN) are null mutations leading to a reduction in the serum PGRN protein levels, missense mutations also have been identifi ed in patients with frontotemporal lobar degeneration and in patients with Alzheimer disease. Among these, p.R564C mutation was identifi ed in a late-onset AD patient with a reduced serum PGRN level. However, recently, we found the p.R564C mutation in a healthy control subject raising doubts whether this is a pathogenic mutation. In this report, we measured the serum PGRN levels in 20 subjects without the p.R564C mutation and in one subject with the p.R564C mutation, to determine whether the p.R564C mutation is associated with reduced serum PGRN levels. We found that the serum PGRN level in the subject with the p.R564C mutation was not reduced compared to the subjects without the p.R564C mutation. Our result reiterates that p.R564C may not be a pathogenic mutation.

Infantile digital fibromatoais is a rare benign tumor on fingers and toes of infants and childhood characterized by fibroblaatic proliferation. A 6 months old male infant had a bean sized, flesh nodule on th left 3rd finger. The lesion was noted at 3 days after birth and surgically removed at 7 months old, However, a new lesion developed at the margin of the ald lesion 2 months later. The second patient was a 21 months old female who had two bean sized, flesh nodules on the right 4th snd 5th fingers. The lesion was first noted at 12 months and regressed spontaneously at 30 months without specific interventiona. Histopathological examination of the two cases showed eosinophiric ntracytoplasmic inclusion bodies, charateristic for infantile digital fibromatosis.
Female ; Fibroma* ; Fingers ; Humans ; Inclusion Bodies ; Infant ; Male ; Parturition ; Recurrence ; Toes

OBJECTIVES: The aim of this study is to evaluate the polysomnographic characteristics and prescription status of restless legs syndrome (RLS) patients in naturalistic setting. METHODS: We reviewed medical record of the patients over 18 years olds who (i) satisfied the clinical RLS diagnostic criteria and (ii) had the polysomnography and got treatment related thereto. As a baseline, we evaluated the four diagnostic criteria of the International Restless Legs Syndrome Study Group (IRLSSG) and the International Restless Legs Scale (IRLS) of the subjects. Then the polysomnography and the suggested immobilization test (SIT) were conducted and, after one month of pharmacotherapy using dopamine agonist, the IRLS was evaluated again. RESULTS: A total of 211 subjects participated in this analysis and 94 (44.5%) of them were male and the other 117 (55.5%) were female and the average age of the 211 subjects was 46.9+/-14.2. Out of such 211 subjects, 136 subjects (64.5%) also had the obstructive sleep apnea (OSA), and 53 subjects (25.1%) also had the periodic limb movement disorder (PLMD). 185 subjects (87.7%) out of the 211 subjects had some other sleep disorders except RLS. The results of the polysomnography were as follows : 78.0% of sleep efficiency, 86.8 min of wake after sleep onset, and 3.4% of N3. More specifically, 12.4/h of the average apnea hypopnea index, 14.8/h of the periodic limb movement during sleep (PLMS), 41.2/h of the periodic limb movement during wake during SIT and 21.6/h of total arousal index during sleep. Out of the total subjects, 149 (70.6%) of them took the ropinirole and 47 (22.3%) of them took the pramipexole, and the average dosage of ropinirole was 0.9mg(dosage range 0.125-5 mg) while the average dosage of pramipexole was 0.5 mg (dosage range 0.125-4 mg). The dosage of the ropinirole showed a significant positive correlation with the age (r=0.25, p=0.002) and also with the IRLS (r=0.23, p=0.038). The IRLS at the baseline was 24.9 while the same was decreased down to 13.4 after one month. CONCLUSIONS: Analyzing the result of this study, a majority of clinical RLS subjects demonstrated comorbidity with some other sleep disorder such as the OSA or PLMD. 25.1% of the subjects showed a PLMD, which was less than in previous researches and the average PLMS was not very high as 14.8/h. The dosage of dopamine agonist taken was often a bit more than the amount recommended in Korea. A prospective research using a large scale controlled subjects will be necessary with respect to this topic.
Apnea ; Arousal ; Benzothiazoles ; Comorbidity ; Dopamine Agonists ; Extremities ; Female ; Humans ; Immobilization ; Indoles ; Isothiocyanates ; Korea ; Male ; Medical Records ; Nocturnal Myoclonus Syndrome ; Polysomnography ; Prescriptions ; Restless Legs Syndrome ; Sleep Apnea, Obstructive ; Sleep Wake Disorders

The clinical features of corticobasal degeneration (CBD) are quite asymmetric. The severity of clinical symptoms and dopamine transporter (DAT) bindings were less correlated compared to other parkinsonisms, suggesting that presynaptic nigrostriatal dopaminergic dysfunction may not explain extrapyramidal manifestations in CBD. Therefore we wanted to reexamine asymmetry and severity between DAT imaging and clinical findings. We studied patients meeting the diagnostic criteria for CBD based on clinical features. We collected their clinical information and imaging retrospectively. Seven patients were enrolled and all had asymmetric rigidity, bradykinesia and unilateral limb dystonia. These symptoms did not improve with levodopa. All patients showed symptoms bilaterally in the last visit, but asymmetry of clinical symptoms was remarkable at the time of DAT imaging. The DAT bindings were decreased in six subjects. However, one patient showed normal DAT binding. Four patients had a more evident DAT reduction on the side contralateral to the more clinically affected side, however, two patients had a more prominent reduction on the ipsilateral side. The symptoms that we regard as parkinsonian features in CBD are not only explained by presynaptic dopaminergic dysfunction. Our findings suggest that postsynaptic dopaminergic or nondopaminergic systems may play a major role in parkinsonian symptoms in corticobasal syndrome.
Parkinsonian Disorders

Sporadic spastic paraplegia (SSP) and hereditary spastic paraplegia (HSP) belong to a clinical and genetically heterogeneous group of disorders characterized by progressive spasticity and weakness in the lower extremities. The symptoms are associated with pyramidal tract dysfunction and degeneration of the corticospinal tracts. Parkinsonism is uncommon in SSP/HSP patients. However, both disorders are associated with damage to the nigrostriatal dopaminergic system. In the present study, the clinical features of patients with SSP/HSP were investigated, and nigrostriatal dopaminergic binding potential was assessed using dopamine transporter (DAT) single-photon emission computer tomography (SPECT). Nine patients with spastic paraplegia participated in the present study. The subjects underwent DAT SPECT using the agent [2-[[2-[[[3-(4-chlorophenyl)-8-methyl-8-azabicyclo[3,2,1]oct-2-yl]methyl](2-mercaptoethyl)amino]ethyl]amino]ethanethiolato (3-)-N2,N20,S2,S20]oxo-[IR-(exo-exo)])-[99mTc]technetium ([99mTc]TRODAT-1). The [99mTc]TRODAT-1 SPECT images of five patients appeared normal, whereas the images of four patients revealed reduced striatal ligand uptake. Among the four patients with reduced uptake, two had parkinsonism, and one exhibited periodic limb movements and restless leg syndrome. Our DAT SPECT imaging study shows that reduced DAT density may be observed in patients with parkinsonism. The results of the present study offer an explanation for the spectrum of spastic paraplegia symptoms and the progression of the disorder.
Adult ; Aged ; Brain/*radionuclide imaging ; Dopamine Plasma Membrane Transport Proteins/metabolism ; Female ; Humans ; Male ; Middle Aged ; Organotechnetium Compounds/diagnostic use ; Paraplegia/diagnosis/genetics/*radionuclide imaging ; Parkinsonian Disorders/complications/genetics/*radionuclide imaging ; Pyramidal Tracts ; Radiopharmaceuticals/diagnostic use ; Spastic Paraplegia, Hereditary/diagnosis/genetics/*radionuclide imaging ; Tomography, Emission-Computed, Single-Photon

No abstract available.

Background and Objectives: This study aimed to examine factors of poor outcome by analyzing the outcomes of bilateral subthalamic deep brain stimulation in Parkinson’s disease after 3 years. Methods: We assumed that patients who could not manage independent life in their best stimulationon/medication-on condition after a defi ned period might not have been a good surgical candidate. A poor outcome is defi ned as a failure to maintain functional independence at three years during a stimulation-on/medication-on state. Results: A total of 84 patients underwent bilateral subthalamic deep brain stimulation and all were followed up for 3 years. We excluded one patient who had intracranial hemorrhage. Twenty-one patients of the 83 patients could not keep up independent life even during their best condition for the following reasons: freezing in 15 patients, dementia in 5, depression in 3, musculoskeletal problems in 7, and cancer in one patient. Conclusions: Many patients could not keep up independent life during their best condition as early as three years after deep brain stimulation. Musculoskeletal problems were one major cause of disabilities, as were freezing and dementia. We emphasize that musculoskeletal problems need more attention in the preoperative screening of deep brain stimulation candidates and during the follow up.