Positional Tremor Research Articles

LMNB1-Related Adult-Onset Autosomal Dominant Leukodystrophy Presenting as Movement Disorder: A Case Report and Review of the Literature.

Adult-onset autosomal dominant leukodystrophy (ADLD) is a lately described rare form of leukodystrophy with only one family report from China. As the only disease associated with increased lamina B1 encoded by LMNB1, ADLDs have different clinical presentations, ranging from autonomic to pyramidal tract and cerebellar ataxia. Here, we report a case of ADLD that presented with positional tremor as the initial symptom. T2-weighted brain MRI showed brain atrophy and diffuse high signal intensity of the cerebral white matter and the brain stem. The precise diagnosis was made by identification of the mutated gene. To the best of our knowledge, this is perhaps the first case report of ADLD presenting as tremor in China.

Differential diagnosis of Parkinson and essential tremor with convolutional LSTM networks

This study aims to present a novel method for differentiation of Parkinsonian tremor (PT) and essential tremor (ET) using both postural and resting tremor. A convolutional long short-term memory (LSTM) that learns hand tremor recorded at both postural and resting positions is proposed for differentiation of PT and ET. 3D landmark points of hands at resting and postural positions are gathered using a Leap Motion Controller. A convolutional LSTM is trained for differentiation of ET and PT after preprocessing tremor data. The method is evaluated and tested on a dataset containing 40 subjects where 23 of them had PT and 17 of them had ET. The experiments showed that the accuracy of combined positions using both resting and postural tremor was higher than using single kind of positional tremor. The accuracy of tremor classification is 90% for the combined positions.

Wearable Cardioverter-Defibrillator in a Patient with Left Ventricular Noncompaction/Hypertrabeculation, Coronary Artery Disease, and Polyneuropathy

A 55-year-old Caucasian male with coronary heart disease was admitted because of dyspnea for 4 weeks. Echocardiography showed a dilated left ventricle with an ejection fraction of 34% and apical left ventricular hypertrabeculation/noncompaction with an apical thrombus. Neurologic examination revealed positional tremor and generally reduced tendon reflexes. During 8 weeks, his condition improved under pharmacotherapy. The patient was skeptical about implantable cardioverter-defibrillator (ICD) and expected further improvement from pharmacotherapy. Thus, he received a wearable cardioverter-defibrillator (WCD). We conclude that a WCD might be useful in noncompaction patients in whom improvement of systolic dysfunction is expected or who are skeptical about ICDs.

CADASIL mutation and Balo concentric sclerosis: A link between demyelination and ischemia?

A 26-year-old man of Portuguese descent with no significant past medical history presents with subacute onset of right-sided hemiparesis and aphasia, with marked expressive aphasia, word-finding difficulty, and rare paraphrasic errors. Examination demonstrated right central facial weakness, 2–3/5 strength in the right arm and leg, and dysmetria in the right upper and lower extremities. Reflexes were hyperactive on the right with a right-sided Babinski response. MRI of the brain showed multiple bilateral concentric ring-like structures in the centrum semiovale and the corona radiata on T2 imaging (figure,A), consistent with the pattern of Balo concentric sclerosis. There was associated restricted diffusion in 3 lesions and incomplete ring enhancement in 1 lesion. CSF analysis showed 30 leukocytes (79% lymphocytes, 4% monocytes), erythrocyte count was 850, and oligoclonal bands were absent. He was treated with 1 g of IV methylprednisolone daily for 5 days, with little improvement. He then underwent 5 plasmapheresis exchanges, with significant improvement of motor, sensory, speech deficits, and gait. Forty-five days after discharge, his examination showed 4+/5 strength in the right intrinsic muscles of the hand and a right-sided positional tremor. Gait and tandem gait were normal. He was placed …

Neurobehavioral and clinical effects in workers exposed to CS 2

Objectives Diffuse vascular encephalopathy, parkinsonism and peripheral polyneuropathy (PNP) due to CS 2 exposure are known since long. Data concerning low exposure are sparse. The current study aimed to evaluate whether the decrease of exposure below the threshold limit value (TLV) of 31 mg/m 3 results in an absence of neurological health effects using the same health outcomes as in the first study [Vanhoorne et al., 1995. An epidemiologic study of the effects of carbon disulfide on the peripheral nerves. Int. J. Occup. Environ. Health 1, 295–302]. Methods The study was conducted in a group of workers of a viscose rayon plant and a control group (CG, n = 66 ). The exposed group was divided into two groups, according to the average CS 2 exposure being below (EG1; n = 60 ) or above 31 mg/m 3 (EG2; n = 25 ). If the current TLV seemed insufficient to prevent effects, the analysis was repeated with three subgroups (⩽10, >10 to ⩽30, >30 mg/m 3) to estimate the NOEL. Every participant filled in the neurotoxicity symptom checklist (NSC-60) and the Q16. Clinical neurological examination, computer-assisted neurobehavioral tests (NES) and neurophysiological examinations (nerve conduction velocities and electromyography (EMG)) were performed. Results Sensorimotor complaints (NSC-60) were significantly worse in both EG1 and EG2 compared to CG ( p = 0.032 ) and both exposure groups had a higher proportion of positional tremor compared with CG ( p = 0.046 ). Finger tapping in the dominant and non-dominant hand (NES) was worse in EG1 and EG2 compared with CG (both p < 0.001 ). A significant increase in sural nerve SCV, a significant decrease in sural nerve SNAP amplitude and duration and sympathic skin response (SSR) amplitude, and higher prevalence of EMG abnormalities and PNP was also found in both EG1 and EG2 ( p < 0.003 ). All effects remained significant after controlling for possible confounding factors. Further analysis suggested that even average CS 2 exposures of ⩽10 mg/m 3 could still cause effects on positional tremor, finger tapping dominant and non-dominant hand, sural nerve amplitude, SSR amplitude and EMG abnormalities. Conclusions An excess of psychomotor slowing, tremor and PNP was found in workers on average exposed to CS 2 concentrations below 31 mg/m 3. Data suggest that even average exposures as low as ⩽10 mg/m 3 can cause the deleterious effects. This indicates that the current TLV insufficiently protects against neurological effects and, as a consequence, should be lowered.

Schizophrenieähnliche Symptomatik bei der Westphal-Strümpell-Verlaufsform des Morbus Wilson

Wilson's disease is a rare, autosomal recessive disorder of copper metabolism due to low serum ceruloplasm, resulting in increased copper deposition, especially in the liver and basal ganglia in the brain. The pseudosclerotic type of Wilson's disease, also known as the Westphal-Strümpell form, is distinguished by positional tremor, ataxia and dysarthria as the main symptoms. We use the example of a 23-year-old patient whose neurological symptoms were preceded by a long history of a schizophrenic-like disorder. Clinical symptoms are presented. MRI, SPECT and PET images are illustrated. Therapy and outcome are discussed.