A case of Graves' disease presenting with internal ophthalmoplegia during methylmercaptoimidazole treatment.

A 28-year-old Japanese woman positive for TSH receptor antibody and anti-nuclear antibody complained of difficulty seeing nearby objects, severe throbbing retro-orbital pain, diplopia, blepharoptosis and upward gaze palsy when she became hypothyroid during treatment with 30 mg methylmercaptoimidazole for Graves' hyperthyroidism. Brain magnetic resonance imaging revealed slightly swollen bilateral inferior rectus muscles, suggesting the external ophthalmoplegia due to the muscle pathology commonly encountered in Graves' disease. The retro-orbital pain was associated with marked accommodation failure and the pupillary abnormalities. The left and/or right eye showed intermittent, asymmetric and fluctuating mydriasis, being unresponsive to ordinary light but slowly responsive to strong sunlight and slowly responsive in a dark room. During the 5-year period, mydriasis was observed 9 times on both sides, 11 times only on the right side and 4 times only on the left side. Internal ophthalmoplegia with tonic pupils and accommodation failure affecting both the pupillary sphincter muscle and ciliary muscle due to damage to the parasympathetic outflow to these muscles was suggested. Autoimmune mechanism and/or the mechanism underlying channelopathy affecting the ciliary ganglion or short ciliary nerves might be responsible for this fluctuating complication. This very rare panophthalmopathy affecting both external and internal muscles occurred when the patient was suffering from iatrogenic hypothyroidism during the 30 mg methylmercaptimidazole treatment for Graves' disease.

A case of position dependent tremor.

A 79-year-old woman presented 3 years' history of hand shaking while drinking a cup of tea. The tremor was seen bilaterally, more predominantly on the left, and it also appeared when reading a book or writing. It was also induced by flexing the elbow to about 90 degrees or more without any specific task. Although there was no family history, the tremor in the present case was clinically diagnosed as essential tremor, because there were no other movement abnormalities, and other causes of tremor were excluded by laboratory tests. The tremor was dependent on the position of the involved extremity regardless of the kind of tasks. Position-specific tremor is discussed in relation to postural tremor.

Evaluation of movement and brain activity.

Clinical neurophysiology studies can contribute important information about the physiology of human movement and the pathophysiology and diagnosis of different movement disorders. Some techniques can be accomplished in a routine clinical neurophysiology laboratory and others require some special equipment. This review, initiating a series of articles on this topic, focuses on the methods and techniques. The methods reviewed include EMG, EEG, MEG, evoked potentials, coherence, accelerometry, posturography (balance), gait, and sleep studies. Functional MRI (fMRI) is also reviewed as a physiological method that can be used independently or together with other methods. A few applications to patients with movement disorders are discussed as examples, but the detailed applications will be the subject of other articles.

Nomenclature of Genetically Determined Myoclonus Syndromes: Recommendations of the International Parkinson and Movement Disorder Society Task Force.

Genetically determined myoclonus disorders are a result of a large number of genes. They have wide clinical variation and no systematic nomenclature. With next-generation sequencing, genetic diagnostics require stringent criteria to associate genes and phenotype. To improve (future) classification and recognition of genetically determined movement disorders, the Movement Disorder Society Task Force for Nomenclature of Genetic Movement Disorders (2012) advocates and renews the naming system of locus symbols. Here, we propose a nomenclature for myoclonus syndromes and related disorders with myoclonic jerks (hyperekplexia and myoclonic epileptic encephalopathies) to guide clinicians in their diagnostic approach to patients with these disorders. Sixty-seven genes were included in the nomenclature. They were divided into 3 subgroups: prominent myoclonus syndromes, 35 genes; prominent myoclonus syndromes combined with another prominent movement disorder, 9 genes; disorders that present usually with other phenotypes but can manifest as a prominent myoclonus syndrome, 23 genes. An additional movement disorder is seen in nearly all myoclonus syndromes: ataxia (n = 41), ataxia and dystonia (n = 6), and dystonia (n = 5). However, no additional movement disorders were seen in related disorders. Cognitive decline and epilepsy are present in the vast majority. The anatomical origin of myoclonus is known in 64% of genetic disorders: cortical (n = 34), noncortical areas (n = 8), and both (n = 1). Cortical myoclonus is commonly seen in association with ataxia, and noncortical myoclonus is often seen with myoclonus-dystonia. This new nomenclature of myoclonus will guide diagnostic testing and phenotype classification. © 2019 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

Corrigendum to "A revised glossary of terms most commonly used by clinical electroencephalographers and updated proposal for the report format of the EEG findings. Revision 2017" [Clin. Neurophysiol. Practice 2 (2017) 170-185].

[This corrects the article DOI: 10.1016/j.cnp.2017.07.002.].

Large fasciculation can clinically manifest as spinal myoclonus; electromyographic and dynamic echomyographic studies of four cases with motor neuron disease.

OBJECTIVE: Patients with motor neuron disease rarely present with fasciculation which is large enough to be clinically recognized as myoclonus. This study is aimed at elucidating the features of large fasciculation manifesting as myoclonus by using surface electromyography (surface EMG) and dynamic echomyography (dynamic Echo). METHODS: Four patients with amyotrophic lateral sclerosis, two of whom clinically presented with both fasciculation and myoclonus, were studied by using the surface EMG and the dynamic Echo. RESULTS: At rest, all patients had fasciculation in atrophic muscles, and the surface EMG showed occasional discharges of different waveforms corresponding to fasciculation. During voluntary gentle muscle contraction, the surface EMG showed repetitive discharges in the contracting muscle, which were constant in size and waveform within each muscle. The muscle Echo at rest revealed occasional contractions of a small number of muscle fibers corresponding to fasciculation. During voluntary muscle contraction, the number of muscle fibers involved in the involuntary motor phenomena was larger in the patients who clinically presented with myoclonus compared with other patients who clinically presented only with fasciculation. In a patient who presented with myoclonus, there was no contraction in the antagonist muscle. CONCLUSIONS: Fasciculation involving a large number of muscle fibers clinically manifests as spinal myoclonus. SIGNIFICANCE: Fasciculation involving a large number of muscle fibers can be a cause of spinal myoclonus.

Identification and field attraction of the female sex pheromone of a kiwifruit pest, Nokona feralis (Lepidoptera: Sesiidae).

Female sex pheromone of a clearwing moth Nokona feralis (Leech) (Lepidoptera: Sesiidae), a pest of kiwifruit, was identified to be a 7:3 mixture of (3E,13Z)-3,13-octadecadienyl acetate (E3,Z13-18:OAc) and (3E,13Z)-3,13-octadecadien-1-ol (E3,Z13-18:OH) by GC-EAD and GC/MS analyses. Males were attracted to wide-range mixtures of E3,Z13-18:OAc and E3,Z13-18:OH, and a 7:3 mixture of those two compounds strongly attracted the males in the field.

[A Case of Cowden Syndrome Associated with Lhermitte-Duclos Disease].

A 24-year-old woman slowly developed mild unsteadiness of gait. Neurological examination revealed mild dysmetria of the left upper and lower limbs. Standing and gait were unsteady, and tandem gait was impossible. Cranial magnetic resonance imaging (MRI) showed an enlarged left cerebellar hemisphere with striated lines, a characteristic finding of Lhermitte-Duclos disease. She also had papules on the forehead, goiter, lactating adenoma, glycogenic acanthosis in the esophagus, café-au-lait spot, and hemangioma and keratosis on the dorsum of foot. The diagnosis of Cowden syndrome was established by finding the mutation in the phosphatase and tensin homolog deleted on chromosome 10 (PTEN) gene. Cowden syndrome is an autosomal dominant disorder characterized by multiple hamartomas in a variety of tissues. Recognition of Lhermitte-Duclos disease as a neurological condition of Cowden syndrome is important, and once the diagnosis of Lhermitte-Duclos disease is made, a close physical investigation is necessary because the hamartomas tend to develop malignancies. (Received March 15, 2017; Accepted July 24, 2017; Published December 1, 2017).

A case of bulbospinal muscular atrophy with large fasciculation manifesting as spinal myoclonus.

OBJECTIVE: This paper reports a patient with bulbospinal muscular atrophy (BSMA) who presented with spinal myoclonus, documented by video and surface electromyography. CASE REPORT: A 66-year-old man had progressive gait disturbance, dysphagia, and easy fatigability of all extremities over a period of 4 years. Neurologically, muscle atrophy, fasciculation, and weakness were observed in the bulbar and limb muscles. When the knees were kept in mild flexion in the supine position, fasciculation of the thigh adductor muscles was so large that it caused shock-like involuntary movements of the legs, corresponding to spinal myoclonus. A genetic test revealed 41 repeats of CAG in the androgen receptor gene, and the diagnosis of BSMA was made. SIGNIFICANCE: The present case suggests that extremely large fasciculation can cause spinal myoclonus.

Neurophysiology of myoclonus and progressive myoclonus epilepsies.

The high temporal resolution of neurophysiological recordings makes them particularly suited to faithfully describing the time course of rapid events such as myoclonus and to precisely measure its time relationship with other related activities. In progressive myoclonus epilepsies (PMEs) polygraphy with simultaneous EMG-EEG recordings is a crucial tool for defining the characteristic of myoclonic jerks their topography over different muscles (namely antagonists), their time course and relationship with vigilance muscle activation and stimulations. Moreover on polygraphic recordings it is possible to detect EEG activities associated to myoclonic jerks and define their time relationship with myoclonus thus differentiating cortical types of myoclonus from subcorticallly generated ones. Tanks to the back averaging technique non obvious time-locked EEG potentials can be detected on polygraphy , furthermore in stimulus sensitive myoclonus the analysis can include the potential evoked by the somatosensory stimulus (SEP). The polygraphic recording also gives information on muscle activity suppression occurring after jerk or as pure negative myoclonus. Besides the time domain analysis, techniques based on frequency analysis have been developed to evaluate EEG-EMG coherence. The neurophysiological techniques provide investigators and clinicians with an invaluable information to define the type of myoclonus and its generating circuitry thus substantially contributing in the diagnosis and management of PMEs.

[A case of MELAS associated with histochemical findings of muscles characteristic of MERRF].

We here report a 39-year-old woman of short stature with sensorineural deafness, who suddenly developed status epilepticus. T2-weighed image of brain magnetic resonance imaging (MRI) revealed a high signal lesion in the left temporal area, the distribution of which was not compatible with any particular arterial supply. Lactate and pyruvate were elevated in the serum and cerebrospinal fluid. As the mitochondrial gene analysis revealed the m.3243A>G mutation, diagnosis of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode (MELAS) was made. In the histochemical study of a biopsied muscle, the intramuscular blood vessels reacted strongly with SDH (SSV), but the SSV was negative for cytochrome c oxidase (COX), the findings characteristic of myoclonic epilepsy with ragged-red fibers (MERRF). This is the first case of MELAS in which the muscle histochemistry showed positive SSV unassociated with increased COX.

Parkinson's disease patients showed delayed awareness of motor intention.

Although dopamine plays an important role for motor control and modulates the frontal function via basal ganglia-thalamo-cortical loop, it is not known whether dopamine can affect the awareness of motor intention or not. To test this hypothesis, we applied Libet's clock paradigm to Parkinson's disease (PD) patients. Thirteen PD patients and 13 age-matched, healthy controls took part in the experiment which consisted of three judgment paradigms: W, M and S judgment. In W and M judgments, subjects were asked to press the key at self-willed timing. In W judgment, subjects reported the location of the clock's hand when they became aware of the intention (W-time). In M judgment, subjects reported the time when they became aware of the actual movement (M-time). In S judgment, subjects reported the time of the electrical stimulation given to their hand (S-time). W-time was significantly shorter in PD patients than in healthy control subjects while M-time and S-time were not different between the two groups. Delayed awareness of motor intention but not of action in PD patients might be related to dopamine depletion in those patients.

Automatic interpretation and writing report of the adult waking electroencephalogram.

Automatic interpretation of the EEG has so far been faced with significant difficulties because of a large amount of spatial as well as temporal information contained in the EEG, continuous fluctuation of the background activity depending on changes in the subject's vigilance and attention level, the occurrence of paroxysmal activities such as spikes and spike-and-slow-waves, contamination of the EEG with a variety of artefacts and the use of different recording electrodes and montages. Therefore, previous attempts of automatic EEG interpretation have been focussed only on a specific EEG feature such as paroxysmal abnormalities, delta waves, sleep stages and artefact detection. As a result of a long-standing cooperation between clinical neurophysiologists and system engineers, we report for the first time on a comprehensive, computer-assisted, automatic interpretation of the adult waking EEG. This system analyses the background activity, intermittent abnormalities, artefacts and the level of vigilance and attention of the subject, and automatically presents its report in written form. Besides, it also detects paroxysmal abnormalities and evaluates the effects of intermittent photic stimulation and hyperventilation on the EEG. This system of automatic EEG interpretation was formed by adopting the strategy that the qualified EEGers employ for the systematic visual inspection. This system can be used as a supplementary tool for the EEGer's visual inspection, and for educating EEG trainees and EEG technicians.

Automatic reference selection for quantitative EEG interpretation: identification of diffuse/localised activity and the active earlobe reference, iterative detection of the distribution of EEG rhythms.

EEG (Electroencephalograph) interpretation is important for the diagnosis of neurological disorders. The proper adjustment of the montage can highlight the EEG rhythm of interest and avoid false interpretation. The aim of this study was to develop an automatic reference selection method to identify a suitable reference. The results may contribute to the accurate inspection of the distribution of EEG rhythms for quantitative EEG interpretation. The method includes two pre-judgements and one iterative detection module. The diffuse case is initially identified by pre-judgement 1 when intermittent rhythmic waveforms occur over large areas along the scalp. The earlobe reference or averaged reference is adopted for the diffuse case due to the effect of the earlobe reference depending on pre-judgement 2. An iterative detection algorithm is developed for the localised case when the signal is distributed in a small area of the brain. The suitable averaged reference is finally determined based on the detected focal and distributed electrodes. The presented technique was applied to the pathological EEG recordings of nine patients. One example of the diffuse case is introduced by illustrating the results of the pre-judgements. The diffusely intermittent rhythmic slow wave is identified. The effect of active earlobe reference is analysed. Two examples of the localised case are presented, indicating the results of the iterative detection module. The focal and distributed electrodes are detected automatically during the repeating algorithm. The identification of diffuse and localised activity was satisfactory compared with the visual inspection. The EEG rhythm of interest can be highlighted using a suitable selected reference. The implementation of an automatic reference selection method is helpful to detect the distribution of an EEG rhythm, which can improve the accuracy of EEG interpretation during both visual inspection and automatic interpretation.

Role of posterior parietal cortex in reaching movements in humans: clinical implication for 'optic ataxia'.

OBJECTIVE: To clarify the spatio-temporal profile of cortical activity related to reaching movement in the posterior parietal cortex (PPC) in humans. METHODS: Four patients with intractable partial epilepsy who underwent subdural electrode implantation were studied as a part of pre-surgical evaluation. We investigated the Bereitschaftspotential (BP) associated with reaching and correlated the findings with the effect of electrical stimulation of the same cortical area. RESULTS: BPs specific for reaching, as compared with BPs for simple movements by the hand or arm contralateral to the implanted hemisphere, were recognized in all patients, mainly around the intraparietal sulcus (IPS), the superior parietal lobule (SPL) and the precuneus. BPs near the IPS had the earlier onset than BPs in the SPL. Electrical stimulation of a part of the PPC, where the reach-specific BPs were recorded, selectively impaired reaching. CONCLUSIONS: Intracranial BP recording and cortical electrical stimulation delineated human reach-related areas in the PPC. SIGNIFICANCE: The present study for the first time by direct cortical recording in humans demonstrates that parts of the cortices around the IPS and SPL play a crucial role in visually-guided reaching.

[A case of myasthenia gravis presenting solely with bulbar palsy unassociated with easy fatigability].

A 69-year-old Japanese female was admitted because of progressive nasal voice and dysphagia. Neurological examination revealed paresis of the soft palate with marked dysphagia and rhinolalia. Otherwise there was no weakness or easy fatigability in extraocular muscles and extremities. On laboratory test, anti-acetylcholine receptor antibody (anti-AChR Ab) was positive, while anti-muscle-specific tyrosine kinase antibody (anti-MuSK Ab) was negative. Edrophonium test was positive, resulting in clear improvement in phonation and swallowing. Harvey-Masland test of ocular and extremity muscles did not show any waning. With the diagnosis of bulbar myasthenia gravis, the patient was treated with methylprednisolone and pyridostigmine, resulting in clear improvement of the symptoms. The present case shows that it is important to consider MG even in cases presenting solely with progressive bulbar palsy without easy fatigability. So far, cases of bulbar myasthenia gravis with positive anti-MuSK Ab have often been reported. As shown in the present case, bulbar myasthenia gravis can also be associated with positive anti-ACh-R Ab but negative anti-MuSK Ab.

Identification of the sex pheromone secreted by a nettle moth, Monema flavescens, using gas chromatography/fourier transform infrared spectroscopy.

The nettle moth Monema flavescens (Limacodidae) is a defoliator of fruit trees, such as Chinese plum and persimmon. The larvae of this species have spines containing a poison that causes serious irritation and inflammation in humans. Coupled gas chromatography-electroantennogram detection and gas chromatography/mass spectrometry analyses of a crude pheromone extract, combined with derivatization, indicated that female moths produced 8-decen-1-ol and 7,9-decadien-1-ol at a ratio of approximately 9:1. The E configuration of the double bonds was assigned for both components from infrared spectra, recorded on a gas chromatograph/Fourier transform-infrared spectrophotometer equipped with a zinc selenide disk cooled to -30 °C. The monoenyl and dienyl alcohols had absorptions characteristic of E geometry at 966 and 951 cm(-1), respectively. A band chromatogram at 951 cm(-1) was useful for distinguishing geometric isomers, because terminal conjugated diene are difficult to resolve, even on high polarity columns. Furthermore, we identified the Z configuration of the same 7,9-dienyl alcohol secreted by another nettle moth, Parasa lepida lepida, through the absence of this absorption. In field trials, lures baited with a 9:1 mixture of (E)-8-decen-1-ol and (E)-7,9-decadien-1-ol attracted M. flavescens males. Furthermore, the field trials indicated that contamination with the (Z)-diene reduced catches to the pheromone mixture more than did contamination with the (Z)-monoene.

Female sex pheromone secreted by Carmenta mimosa (Lepidoptera: Sesiidae), a biological control agent for an invasive weed in Vietnam.

Larvae of the clearwing moth, Carmenta mimosa (Lepidoptera: Sesiidae), bore into the trunk of Mimosa pigra L., which is one of the most invasive weeds in Vietnam. GC-EAD and GC-MS analyses of a pheromone gland extract revealed that the female moths produced (3Z,13Z)-3,13-octadecadienyl acetate. A lure baited with the synthetic acetate alone successfully attracted C. mimosa males in a field test. While the addition of a small amount of the corresponding alcohol did not strongly diminish the number of captured males, a trace of the aldehyde derivative or the (3E,13Z)-isomer markedly inhibited the attractiveness of the acetate. The diurnal males were mainly attracted from 6:00 am to 12:00 am.