Clinical analysis of nine cases of paroxysmal exercise-induced dystonia.

This study was aimed to analyze the clinical features of paroxysmal kinesigenic dyskinesia (PKD) and extend the understanding of this disease. From August, 2008 to October, 2010, 9 patients were diagnosed with PKD in the Department of Neurology of the First Affiliated Hospital of Zhejiang University, China. The data involving clinical demographic characteristics, somatosensory evoked potentials, results of electromyography, video electroencephalography (EEG), brain magnetic resonance imaging (MRI) and computerized tomography (CT) were collected. All PKD patients exhibited unilateral or bilateral recurrent episodic dyskinetic attacks triggered by sudden voluntary movements. The duration of the attacks ranged from several seconds to one minute. The attack frequency ranged from approximately once in several months to more than 10 times in a day. Patients suffered from no conscious disorders during the attack, and no neurological signs were found during the period between attacks. No abnormal somatosensory evoked potentials were found. Routine EEG, video EEG monitoring or brain imaging showed normal findings. Classical treatment for anti-epilepsy, including carbamazepine and topiramate, was administered to the patients and proved to be effective. It was concluded that PKD is characteristically triggered by sudden voluntary movement; no abnormal electroneurophysiological findings are observed in PKD, and antiepileptic drugs are effective in treating the disorder.

[Role of activated brain regions during tactile stimulation in early Parkinson's disease: a preliminary fMRI study].

OBJECTIVE: To investigate the role of activated brain regions in Parkinson's disease (PD) during tactile stimulation. METHODS: Twenty-one patients with early PD[mean age (60.43 +/-9.65)y] and twenty-two age-matched healthy controls [mean age (59.23 +/-11.12)y] were enrolled in the study. All the patients were tested by the United Parkinson Disease Rating Scale (UPDRS) as the evaluation of the disease severity. A block design was used when the finger tactile stimulation was given to the subjects. The hypoactive and hyperactive regions of PD patients were confirmed first, which were identified as regions of interest (ROI). ROI analysis was performed to quantify BOLD signal changes when subjects were under tactile stimulation. The correlations of signal changes with disease severity, and correlations of hyperactive with hypoactive regions were analyzed. RESULTS: Right primary sensory and motor cortex, right supplementary motor area (SMA), bilateral caudates, bilateral precuneus, bilateral occipital visual cortex and left middle temporal gyrus were hypoactivated in PD, while right prefrontal cortex (PFC) and right caudate were hyperactivated. The hypoactivation of right SMA was negatively correlated with disease severity. All the hypoactive and hyperactive regions were positively correlated with activation of caudates. There was a positive correlation between hyperactive PFC and hypoactive regions. CONCLUSIONS: The signal change of SMA is directly related to disease severity in early PD, and caudates may play a significant role in PD tactile processing. The hyperactivation of PFC may be not a compensation but a pathophysiological change related to PD neural dysfunction.

Early second-line therapy is associated with improved episodic memory in anti-NMDA receptor encephalitis.

OBJECTIVE: To investigate whether the early administration of intravenous second-line immunotherapy correlates with improved long-term cognition and the potential mechanisms via imaging in adult patients with moderate-to-severe anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. METHODS: Sixteen adult patients with moderate-to-severe anti-NMDA receptor encephalitis past the acute stage and 15 healthy controls (HCs) performed a set of comprehensive neuropsychological tests, and underwent a resting-state fMRI study to analyze resting state functional connectivity (FC). In addition, correlation analyses were performed between hippocampal FC and cognitive performance. All patients were received intravenous first-line immunotherapy, and nine of them were also given intravenous second-line immunotherapy within 3 months of disease onset. RESULTS: The patients who only received first-line immunotherapy showed significant verbal episodic memory impairments compared with HCs and those who received second-line immunotherapy, while no significant differences were noted between the patients with second-line immunotherapy and the HCs. In line with the results of neuropsychological tests, significant changes in bilateral hippocampal FC were observed in the patients who only received first-line immunotherapy compared with both HCs and those who received second-line immunotherapy. However, no significant differences in hippocampal FC were observed in the patients with second-line immunotherapy compared with the HCs. Importantly, hippocampal-medial prefrontal cortex (mPFC) connectivity positively correlated with memory performance. INTERPRETATION: In the long term, early administration of intravenous second-line immunotherapy may be associated with more favorable verbal episodic memory outcomes in patients with moderate-to-severe anti-NMDA receptor encephalitis. These results may provide some evidence and guidance for the use of immunotherapy in this population.

Asymptomatic syringomyelia accompanied with metastatic cerebellar and spinal intramedullary lymphoma: A case report.

RATIONALE: Asympotamic syringomyelia accompanied with metastatic cerebellar and thoracic spinal intramedullary lymphoma is rare in clinical practice. If the intramedullary lymphoma is large enough, the patient will rapidly develop neurologic signs of spinal injury. The prognosis of this type of complication is always bad. PATIENT CONCERNS: Rapid and correct diagnosis and treatment is important for metastatic extranodal lymphoma with B cell of origin. DIAGNOSES: Syringomyelia accompanied with metastatic cerebellar and thoracic spinal intramedullary lymphoma. INTERVENTIONS: The patient was treated with a combination of systemic chemotherapy and focal radiotherapy and intrathecal therapy. OUTCOMES: Resolution of metastatic lymphoma was not continued after conservative medical management and the patient died finally due to multiple organ failure. LESSONS: Syringomyelia can develop due to the metastatic thoracic intramedullary lymphoma in patients with diffuse malignant large B cell lymphoma. Early and accurate diagnosis, anti-lymphoma treatment, and timely neurosurgical intervention may delay the development of the disease.

Extreme delta brush guides to the diagnosis of anti-NMDAR encephalitis.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a treatable but often misdiagnosed autoimmune encephalitis. Diagnosis depends on NMDAR antibody testing, which may not be readily available. Alternatively, the electroencephalogram (EEG) extreme delta brush pattern may provide a valuable immediate indicator for the diagnosis of anti-NMDAR encephalitis. A 32-year-old female (case 1) presented with fever, headache, behavioral changes, confusion, intractable seizures, central hypoventilation, dysautonomia, facial and limb dyskinesias, and comorbid ovarian teratoma. Cerebral spinal fluid (CSF) testing revealed mild lymphocytic pleocytosis while brain MRI results were normal. A 45-year-old male (case 2) presented with major behavioral changes and rare seizures. Results of routine CSF testing and brain MRI scanning were unremarkable. In both cases, EEG initially revealed the extreme delta brush (EDB) pattern of beta bursting on the peaks and/or the troughs of delta waves, which led to subsequent NMDAR antibody testing and the confirmative diagnosis. Thus, EDB may be a readily accessible sign for suspected anti-NMDAR encephalitis.

TCD study of hemodynamic changes in PCA response to photic stimulation.

OBJECTIVES: During visual stimulation, the elevated metabolism rate will couple with increase of blood flow velocity(BFV) in posterior cerebral artery(PCA). This study with TCD was aimed to investigate whether the coupling might change according to the different vasoneuronal conditions. METHODS: Ninety-nine volunteers including 24 hypertension(HT) patients and 2 patients suffering from both HT and diabetes mellitus (DM) were enrolled in this trial. BFV and pulse indexes(PI) in P2 segments of PCA on both sides were monitored during visual stimulation. RESULTS: In all subjects, Mean BFV increased and PI went down in response to visual stimulation. The percentages of changes (deltaV and deltaP) of both mean BFV and PI were larger in young group( < 55 years old) than in old one(> or = 55 years old). There was significant positive correlation between deltaV and deltaP. Multivariated regression analysis did not show HT and DM, but age related to deltaV(deltaP). We did not find significant difference of deltaV(deltaP) between left and right sides. CONCLUSIONS: Blood flow velocity in PCA P2 segment increased due to decreased cerebrovascular resistance during visual stimulation and the response weakened with aging of the patient.

MRS study on lentiform nucleus in idiopathic Parkinson's disease with unilateral symptoms.

OBJECTIVE: To investigate changes in magnetic resonance spectroscopy (MRS) of lentiform nucleus during the early stage of Parkinson's disease. METHODS: Twenty-five patients with idiopathic Parkinson disease with unilateral symptoms (IPDUS) and 25 healthy volunteers were enrolled in this study. MRS of the lentiform nucleus in each patient was taken and then concentrations of N-acetylaspartate (NAA), Creatine (Cr) and Choline (Cho) were calculated. RESULTS: Compared to that in the control, NAA/ (Cho+Cr) was significantly lower in the lentiform nucleus contralateral to symptoms and even that in the ipsilateral side in IPDUS patients (all P<0.05); while there was no difference between the two sides in the healthy volunteer (P>0.05). The ratio of NAA/(Cho+Cr) ipsilateral to the sympatomatic side of the patient was also lower than that of the control (P<0.05). CONCLUSIONS: There might be some changes with MRS on the lentiform nucleus during the early stage of idiopathic Parkinson's disease with unilateral symptom. MRS may be one of the reliable methods for early or even sub-clinical diagnosis.

[Dynamical examination of auditory event-related potentials P300 and sympathetic skin response in people with insomia of Sweet Dream Capsule therapy].

OBJECTIVE: To observe the change of auditory event-related potentials (P300) and sympathetic skin response (SSR) in people with insomia of Sweet Dream Capsule therapy. METHODS: 30 patients meeting criteria for primary insomnia and 30 healthy volunteers with age matching controls were selected for the study. P300 and SSR were measured before treatment of Sweet Dream Capsule and at week 4 , 8 of the therapeutic course. That the change of P300 and SSR before and after treatment and their relations with PSQI were studied. RESULT: Compared with those of normal controls, both P300 latency and SSR latency were prolonged while amplitude was decreased in patients with insomnia (P < 0.01). P300 amplitude was increased significantly at central (Cz) electrode sites only at week 8 when compared with amplitude before treatment (P < 0.05). With improvement of symptom and PSQI scores, latency and amplitude of SSR were improved at week 4 and week 8 (P < 0.05 and P < 0.01) . CONCLUSION: P300 has a some improvement in people with insomia of Sweet Dream Capsule therapy while SSR im proves significantly, and PSQI scores are ameliorated too.

Altered activation in visual cortex: unusual functional magnetic resonance imaging finding in early Parkinson's disease.

OBJECTIVE: Movement dysfunction in Parkinson's disease (PD) is well documented but its cause remains unclear. This study examined whether motor symptoms are caused by sensory impairment, executive dysfunction or other factors. METHODS: We examined the integration of tactile and motor programmes using functional magnetic resonance imaging (fMRI) in early stage PD patients compared with neurologically normal controls, to investigate neural mechanisms underlying movement dysfunction. RESULTS: Twenty-two control participants exhibited activation in an extensive network of cortical regions involved in tactile-motor integration. Decreased activation in somatosensory and motor regions during performance of passive tactile and movement tasks was found in 21 PD patients. The extrastriate visual cortex exhibited greater activation in controls during performance of the tasks. In contrast, greater activation in bilateral prefrontal regions was found in PD patients. CONCLUSIONS: These results indicate that the extrastriate visual cortex is a multisensory region playing an important role in sensorimotor integration. The findings suggest that PD patients exhibit decreased activation in the extrastriate visual cortex, possibly related to dysfunctional integrative processing, and compensatory activity in the prefrontal cortex.

Changes in spontaneous brain activity in early Parkinson's disease.

Resting state brain activity can provide valuable insights into the pathophysiology of Parkinson's disease (PD). The purpose of the present study was (a) to investigate abnormal spontaneous neuronal activity in early PD patients using resting-state functional MRI (fMRI) with a regional homogeneity (ReHo) method and (b) to demonstrate the potential of using changes in abnormal spontaneous neuronal activity for monitoring the progression of PD during its early stages. Seventeen early PD patients were assessed with the Unified Parkinson's Disease Rating Scale (UPDRS), the Hoehn and Yahr disability scale and the Mini-mental State Examination (MMSE) were compared with seventeen gender- and age-matched healthy controls. All subjects underwent MRI scans using a 1.5T General Electric Signa Excite II scanner. The MRI scan protocol included whole-brain volumetric imaging using a 3D inversion recovery prepared (IR-Prep) fast spoiled gradient-echo pulse sequence and 2D multi-slice (22 axial slices covering the whole brain) resting-state fMRI using an echo planar imaging (EPI) sequence. Images were analyzed in SPM5 together with a ReHo algorithm using the in-house software program REST. A corrected threshold of p<0.05 was determined by AlphaSim and used in statistical analysis. Compared with the healthy controls, the early PD group showed significantly increased ReHo in a number of brain regions, including the left cerebellum, left parietal lobe, right middle temporal lobe, right sub-thalamic nucleus areas, right superior frontal gyrus, middle frontal gyrus (MFG), right inferior parietal lobe (IPL), right precuneus lobe, left MFG and left IPL. Additionally, significantly reduced ReHo was also observed in the early PD patients in the following brain regions: the left putamen, left inferior frontal gyrus, right hippocampus, right anterior cingulum, and bilateral lingual gyrus. Moreover, in PD patients, ReHo in the left putamen was negatively correlated with the UPDRS scores (r=-0.69). These results indicate that the abnormal resting state spontaneous brain activity associated with patients with early PD can be revealed by Reho analysis.