Anterior disc displacement is a common subtype seen in temporomandibular disorders (TMD) patients. It may cause mandibular movement disorders, such as clicking of joint, intermittent closed lock, limitation of mouth opening, etc. These disorders may affect the life qualities of patients. Anterior disc displacement may also cause mandibular malformations, especially among adolescents, which may affect the growth of condyle, therefore may have a correlation with mandibular retrusion or mandibular deviation when grown up. This paper going to review the influences of anterior disc displacement on oral mandibular function and morphology and their biological mechanisms.
Adolescent ; Humans ; Mandible ; abnormalities ; pathology ; Mandibular Condyle ; growth & development ; Mandibular Diseases ; etiology ; Movement ; Retrognathia ; etiology ; Temporomandibular Joint Disorders ; etiology ; physiopathology

OBJECTIVETo investigate the clinical, biochemical and genetic profiles of 28 Chinese patients with glutaric aciduria type 1.

METHODTwenty-eight patients with glutaric aciduria type 1 seen in the Department of Pediatrics, Peking University First Hospital from July 2003 to October 2013 were studied. The data of clinical course, laboratory examinations, cranial MRI and GCDH gene mutations of the patients were analyzed.

RESULT(1) Three cases were detected by newborn screening, and the other patients were diagnosed at the age of 2 months to 17 years. (2) 22 patients (79%) were infant onset cases with psychomotor retardation, dystonia, seizures, athetosis, recurrent vomiting, drowsiness or feeding difficulty. Only two of the 22 patients with infant onset got normal intelligence and movement after treatment. Twenty of them were improved slowly with delayed development, dystonia and other neurological problems. Three patients (11%) had late onset. They had motor regression, headache and seizure at the age of 8, 9 and 17 years, respectively. Rapid improvement was observed after treatment. (3) Cranial MRI has been checked in 23 patients; 22 of them showed characteristic widening of the Sylvian fissure, abnormalities of the basal ganglia, leukoencephalopathy and brain atrophy. Thirty-five mutations in GCDH gene of the patients were identified; c.148T>C (p.W50R) was the most common mutation with the frequency of 7.7%; 6 mutations (c.628A>G, c.700C>T, c.731G>T, c.963G>C, c.1031C>T and c.1109T>C) were novel.

CONCLUSIONGlutaric aciduria type 1 usually induced neurological deterioration resulting in severe psychomotor retardation and dystonia. Most of our patients were clinically diagnosed. Patients with early onset usually remained having neurological damage. Phenotype and genotype correlation has not been found in the patients. Neonatal screening for organic acidurias should be expanded in China.

Age of Onset ; Amino Acid Metabolism, Inborn Errors ; diagnosis ; genetics ; metabolism ; Brain Diseases, Metabolic ; diagnosis ; genetics ; metabolism ; DNA Mutational Analysis ; Follow-Up Studies ; Gas Chromatography-Mass Spectrometry ; Glutarates ; urine ; Glutaryl-CoA Dehydrogenase ; deficiency ; genetics ; metabolism ; Humans ; Infant, Newborn ; Intellectual Disability ; etiology ; pathology ; Magnetic Resonance Imaging ; Movement Disorders ; etiology ; pathology ; Mutation ; Neonatal Screening ; methods ; Retrospective Studies

OBJECTIVETo investigate the high-risk factors for the quality of general movements (GMs), which has a predictive value for brain dysfunction in infants.

METHODSA total of 618 infants in the stage of writhing movements and 539 infants in the stage of fidgety movements were selected separately for the evaluation of GMs. The high-risk factors for the quality of GMs in infants were analyzed by ANOVA, chi-square test, and multivariate logistic regression.

RESULTSMultivariate logistic regression analysis showed that the factors significantly associated with the quality of GMs in the stage of writhing movements were gestational age (OR=0.762, P<0.001), birth weight (OR=0.264, P<0.001), severe asphyxia (OR=2.445, P=0.012), and intrauterine distress (OR=4.865, P<0.001); the factors significantly associated with the quality of GMs in the stage of fidget movements were gestational age (OR=0.786, P=0.003), birth weight (OR=0.217, P<0.001), severe asphyxia (OR=3.765, P=0.001), and hyperbilirubinemia (OR=2.640, P=0.028).

CONCLUSIONSLow gestational age, low birth weight, severe asphyxia, hyperbilirubinemia and intrauterine distress are high-risk factors for abnormal GMs in infants, and early screening and intervention should be performed to reduce the incidence of abnormal nervous system sequelae.

Asphyxia ; complications ; Birth Weight ; Female ; Gestational Age ; Humans ; Infant ; Logistic Models ; Male ; Movement ; Movement Disorders ; etiology ; Risk Factors

OBJECTIVETo study the clinical and laboratory features and diagnosis of the patient with anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis in children.

METHODThe data of clinical feature, laboratory findings, and radiological manifestation were reviewed and analyzed.

RESULTOf the 7 patients, 4 were female and 3 were male. The age of onset was from 6.6 to 15.5 years (average 9.5 years). The onset of 4 cases started with convulsion. Six cases had seizures which was difficult to control by antiepileptic drugs. All patients had psychiatric symptoms and speech disorder. Six cases had different levels of decreased consciousness and dyskinesias. 6 cases had autonomic nerve instability, and 7 cases developed sleep disorders. The results of MRI examination were normal in all patients. The EEG of most patients showed focal or diffuse slow waves. Six cases had oligoclonal bands. All cases were confirmed to have the disease by detection of anti-NMDA receptor antibodies. No tumor was detected in any of the patients. All patients received immunotherapy.

CONCLUSIONAnti-NMDAR encephalitis is a severe but treatable disorder that frequently affects children and adolescents. Pediatric patients had clinical manifestations similar to those of adult patients. But children have a lower incidence of tumors and hypoventilation also occurs less frequently in children. Most of children had a good prognosis.

Adolescent ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; complications ; diagnosis ; therapy ; Autoantibodies ; blood ; cerebrospinal fluid ; Autonomic Nervous System ; physiopathology ; Brain ; diagnostic imaging ; pathology ; Child ; Electroencephalography ; Female ; Humans ; Immunotherapy ; methods ; Magnetic Resonance Imaging ; Male ; Movement Disorders ; etiology ; Radiography ; Receptors, N-Methyl-D-Aspartate ; immunology ; Retrospective Studies ; Seizures ; etiology

OBJECTIVETo explore the relationship of motor dysfunction of the lower extremities with the imaging appearances and clinical features of metastatic epidural spinal cord compression (MESCCs).

METHODSFrom July 2006 to December 2007, 26 successive patients with metastases of the thoracic, lumbar and the cervical spine were treated in our department. Forty-three main involved vertebra in all 26 patients were evaluated by magnetic resonance imaging and computed tomography, and were scored according motor dysfunction in this study. Fourteen patients (25 vertebrae) had motor dysfunction.

RESULTSAmong 26 patients, 12 cases with visceral metastasis,in which had motor dysfunction in 10 cases; 14 cases without visceral metastasis, in which had motor dysfunction in 4 cases; comparison between two groups, P=0.0079. Among vertebral presence of continuity of 43 main involved vertebrae, 16 vertebrae had motor dysfunction;among vertebral absence of continuity, motor dysfunction occurred in 9 vertebrae, comparison between two groups, P=0.1034. Among vertebral presence of lamina involvement of 43 main involved vertebrae, 11 vertebrae had motor dysfunction; among vertebral absence of lamina involvement, motor dysfunction occurred in 14 vertebrae, comparison between two groups, P=0.020 5. Among vertebral presence of protruding of vertebral posterior wall of 43 main involved vertebrae, 12 vertebrae had motor dysfunction; among vertebral absence of protruding of vertebral posterior wall, 13 vertebrae had motor dysfunction, comparison between two groups, P=0.0334. Among vertebral presence of involvement epidural space of 43 main involved vertebrae, 11 vertebrae had motor dysfunction; among vertebral absence of involvement epidural space, 14 vertebrae had motor dysfunction, comparison between two groups, P=0.003 6. Such factors as age, gender, whether or not received regular chem before admission, back pain degree of metastasis, received regular chem before admission, therapeutic efficacy of primary tumor, number of bony metastases outside spine, number of the main involved vertebrae, level of vertebral metastases location, level of continuous involved vertebrae, vertebral-body involvement, fracture of anterior column, fracture of posterior wall, and pedicle involvement had no effects on incidence of motor dysfunction due to MESCC (P>0.05).

CONCLUSIONMESCC with visceral metastases, lamina involvement, presence of outstanding buttocks sign of posterior wall,involvement epidural space tended to cause symptomatic MESCC. Incidence of continuity of main involved vertebrae occurred more frequently in the CUTS compared with other levels of spine.

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Movement Disorders ; etiology ; Radiography ; Spinal Cord Compression ; diagnostic imaging ; etiology ; Spinal Neoplasms ; complications ; secondary

Brain Stem ; physiology ; Humans ; Infant, Newborn ; Infant, Newborn, Diseases ; etiology ; Movement Disorders ; etiology ; Myoclonus ; etiology ; Reflex, Abnormal ; Reflex, Startle ; Tremor ; etiology

This Stroke Rehabilitation Apparatus uses the electromyography triggered neuromuscular electrical stimulation as the means of the major therapeutics, and the fastigial nucleus stimulation as the means of the assistant therapeutics. This paper introduces the overall structure of the apparatus, the principle of its component, the EMG processing based on local nonlinear projective filtering algorithm and the alternating treatment modes. The therapeutic apparatus has the features of non-invasiveness, safety, convenience and strong alternating capability.
Biofeedback, Psychology ; Electric Stimulation Therapy ; instrumentation ; Electromyography ; methods ; Humans ; Movement Disorders ; etiology ; physiopathology ; rehabilitation ; Psychomotor Performance ; physiology ; Recovery of Function ; Stroke ; physiopathology ; Stroke Rehabilitation

OBJECTIVENumerous studies have described both motor defects and cognitive impairments in several strains of rodents following 3-nitropropionic acid (3-NP) intoxication. In the present study, we investigated spatial recognition memory in Kunming mice that just recovered from motor defects induced by 3-NP.

METHODSMouse model was made by systemic subacute 3-NP treatment, and spatial recognition memory was measured through the Y-maze Test, a simple two-trial recognition test.

RESULTS(1) On day 15 following 3-NP treatment, affected Kunming mice did not show motor defects in the Rotarod test and presented normal gait again. (2) In the following Y-maze test after 1h interval, the percentage (90.0%) of mice showing novel arm preference in 3-NP treatment group was significantly higher than the random chance level (50%), although it was only slightly higher than that (83.3%) in control group. On day 45 after 3-NP treatment, mice failed to choose unfamiliar novel arm as first choice, and the same occured in the control group. (3) For both post-intoxicated (on day 15 and day 45 following 3-NP treatment) and control groups, the duration in the novel arm and the frequency of entering it, were longer and higher compared with familiar start and other arms. For these mice that recently recovered from motor defects following 3-NP intoxication, no spatial memory deficits were observed through Y-maze Test.

CONCLUSIONKunming mice used in our assays might possess resistance to cognitive impairment induced by 3-NP, which is consistent with previous findings in Swiss EPM-M1 mice.

Animals ; Behavior, Animal ; Convulsants ; toxicity ; Male ; Maze Learning ; drug effects ; Memory Disorders ; etiology ; Mice ; Mice, Inbred Strains ; Motor Activity ; drug effects ; Movement Disorders ; etiology ; Nitro Compounds ; toxicity ; Poisoning ; complications ; etiology ; Propionates ; toxicity ; Recovery of Function ; drug effects ; physiology ; Rotarod Performance Test ; Time Factors

OBJECTIVETo determine whether the skilled reaching test is an objective method for evaluating long-term neurological deficits after focal cerebral ischemia in mice.

METHODSIn a reaching box, mice were trained to reach food pellets with their left forelimb through a 0.5 cm slit for 3 weeks. Then focal cerebral ischemia was induced by occluding the right middle cerebral artery, and the percentage of success in obtaining food was observed for 4 weeks. In comparison, the neurological deficit score, the holding angle in an inclined board test, and right turns in a corner test were simultaneously performed. At the end of the experiments, brain infarcts and neuron densities were determined.

RESULTAfter focal cerebral ischemia, the percentage of success in the reaching test was reduced, the right turns in the corner test were increased, the neurological deficit score was increased, and the holding angle in the inclined board test was reduced as well. The holding angle recovered 5 d after ischemia, whereas other 3 indicators remained abnormal until 4 weeks. At the end of the experiments, the brain infarct volumes were increased, and the neuron densities in the cortex, hippocampal CA1 region and striatum were reduced in ischemic mice.

CONCLUSIONThe skill reaching test is an objective and stable method for evaluating long-term neurological deficits after focal cerebral ischemia in mice.

Animals ; Behavior, Animal ; physiology ; Brain ; pathology ; physiopathology ; Brain Ischemia ; complications ; physiopathology ; Cell Count ; Male ; Mice ; Mice, Inbred ICR ; Movement Disorders ; etiology ; physiopathology ; Neurologic Examination ; methods ; Neurons ; pathology ; Psychomotor Performance ; physiology

Dermoid sinuses and meningoceles are seldom encountered in the cervical region. Besides, to the best of our knowledge, the coexistence of these types of congenital abnormalities with recurrent meningitis, as well as with mirror movement, has never been reported before. A 14-year-old female with the diagnosis of recurrent meningitis was referred to our clinic from the Department of Infectious Diseases. She had a cervical meningocele mass that was leaking cerebro-spinal fluid (CSF) and an associated mirror movement symptom. Spina bifida, dermoid sinus and meningocele lesions were demonstrated at the C2 level on computed tomography (CT) and magnetic resonance imaging (MRI). She underwent an operation to remove the sinus tract together with the sac, and at the same time the tethered cord between the sac base and the distal end of the spinal cord was detached. The diagnosis of dermoid sinus and meningocele was confirmed histopathologically. These kinds of congenital pathologies in the cervical region may also predispose the patient to other diseases or symptoms. Herein, a case of meningocele associated with cervical dermoid sinus tract which presented with recurrent meningitis and a rare manifestation of mirror movement is discussed. Neurosurgeons should consider the possible coexistence of mirror movement and recurrent meningitis in the treatment of these types of congenital abnormalities.
Adolescent ; Cervical Vertebrae ; Dermoid Cyst/complications/*pathology/surgery ; Female ; Human ; Magnetic Resonance Imaging ; Meningitis/complications/*pathology ; Meningocele/complications/*pathology/surgery ; Movement Disorders/etiology/pathology ; Recurrence