Motor Automatism Research Articles

The profile of epilepsy and its characteristics in children with neurocutaneous syndromes.

The profile of seizures in neurocutaneous syndromes is variable. We aimed to define the characteristics of epilepsy in children with neurocutaneous syndromes. Cross-sectional study over 18 months at a tertiary care pediatric hospital, including children with neurocutaneous syndromes aged between 1 and 15 years, using the 2017-International League Against Epilepsy classification. In 119 children with neurocutaneous syndromes, 94 (79%) had epilepsy. In eight children with neurofibromatosis one with epilepsy, 5 (62.5%) had generalized motor tonic-clonic seizures, 1 (12.5%) had generalized motor epileptic spasms, 1 (12.5%) had generalized motor automatism, and 1 (12.5%) had a focal seizure. In 69 children with tuberous sclerosis complex with epilepsy, 30 (43.5%) had generalized motor epileptic spasms, 23 (33.3%) had focal seizures, and nine (13.0%) had generalized motor tonic-clonic seizures. In 14 children with Sturge-Weber syndrome with epilepsy, 13 (92.8%) had focal seizures, and 1 (7.2%) had generalized motor tonic seizures. Statistically significant associations were found between epilepsy and intellectual disability (P = 0.02) and behavioral problems (P = 0.00). Profiling seizures in children with neurocutaneous syndromes are paramount in devising target-specific treatments as the epileptogenesis in each syndrome differs in the molecular pathways leading to the hyperexcitability state. Further multicentric studies are required to unravel better insights into the epilepsy profile of neurocutaneous syndromes.

Early infantile epileptic encephalopathy type 16: the new clinical and genetic variant of TBC1D24 gene mutation

Objective: to analyse the clinical and neurophysiological data from a case of early infantile epileptic encephalopathy type 16 in a child with homozygous mutation in TBC1D24 gene. Material and methods . Female child M. aged 1 year and 2 months, with identified mutations in TBC1D24 gene was examined. The whole exome sequencing was performed (Next Generation Sequencing with the Illumina HiSeq 1500 platform, USA). Dynamic video-EEG monitoring was conducted with a “Encephalan-Video” RM-19/26 instrument (“Medicom MTD”, Russia). Results . According to the patient history, on the 22nd day of life, the child developed vocalisms with tonic tension of the limbs. At her 2 months of life, focal myoclonia and myoclono-clonies were noted; at her 4 months – return of tonic seizures with vocalisms followed by eyelid myoclonus, perioral myoclonus, ophthalmoclonia, alternating hemiconvulsions and tonic-clonic seizures. The family history of epilepsy was negative; the parents denied any consanguinity, but admitted a chance of being distant relatives. Neurological examination revealed hypotonic-astatic syndrome and psycho-motor retardation. A video EEG monitoring test detected multiple EEG negative multifocal myoclonic episodes in combination with dystonic hyperkinesia and motor automatism. Although typical epileptiform spike-wave discharges were rare, a focus of low-index spike-wave complexes was identified in the left temporal zone. Therapy with valproates, barbiturates and levetiracetam did not produce any significant effect but benzodiazepines (clonazepam) caused a moderate improvement; a switch to clobazam therapy was then recommended. This case of inherited developmental and epileptic encephalopathy was defined as early infantile epileptic encephalopathy type 16 (OMIM#615338) with autosomal recessive inheritance associated with a previously not described homozygous mutation of the TBC1D24 gene, chr:16:2546775 A>C that caused Tyr209Ser amino acid substitution. Pre-conception testing of the TBC1D24 gene under the IVF condition is recommended to the parents. Discussion . Since 2010, a few variants of early infantile epileptic encephalopathy type 16 caused by homozygous mutation of the TBC1D24 gene have been described in the literature. This clinical case is closer to progressive myoclonic epilepsy with dystonia (PMED), which indicates the nosological autonomy of this form of epilepsy. С onclusion . Children with pharmacoresistant epilepsy and epileptic encephalopathies, as well as those with unusual course of these diseases need genetic assessment with the new generation exom sequencing techniques – such as the “hereditary epilepsy” panel, as well as clinical and full-exom sequencing. All authors contributed equally to this publication.

Study on etiology and clinical course of neonatal seizures and their outcome

Background: The aim was to explore etiology, clinical course of neonatal seizure sand their and outcome in Narayana Medical College Hospital, Nellore, Andhra Pradesh, India.Methods: Retrospective study of 65 neonates from 1 month to 1 year diagnosed with epilepsy between November 2016 to August 2018.Results: Most common type of seizures seen are subtle (40%), followed by multifocalclonic (20%), tonic (21%), focal clonic (9.2%), clonic (7.7%) and myoclonic (1.6%). Most common cause of neonatal seizures was HIE (41.5%), followed by hypoglycemia (13.8%), intracranial bleed (10.8%), septicemia (10.8%), hypocalcemia (7.7%). The morality percentage is high (18.2%) when seizures occurred before 12 hours, when seizures occurred between 24-48 hrs it is 14.3%, and mortality was least when seizures occurred between 2-6 days (12.5%). Seizures are common in stage IIHIE (81.5%) compared to Stage I &Stage III. Seizures with adverse outcome are generalized myoclonic and focal clonic seizures. Focal clonic and focal tonic seizures are most often associated with focal injury. Generalized tonic seizures, motor automatism and some myoclonic seizures are associated with diffuse brain injury. Prolonged seizure activity, seizures lasting for many days, repetitive seizures, and the need for multiple anticonvulsants to control seizure activity are associated with an increased mortality.Conclusions: Authors conclude that prompt recognition, evaluation and treatment of these neonatal events are important in improving the survival of neonates with seizures.

Neonatal seizures: clinical manifestations and etiology

Background Neonatal seizures or fits are signs of central ner-vous system (CNS) diseases, metabolic disorders, or other dis-eases disrupting the CNS. Neonatal seizures are poorly classi-fied, under-recognized and often difficult to treat. It is important torecognize the type of neonatal seizures that might be the only signof a CNS disorder.Objective To recognize the type and etiology of neonatal sei-zures in several hospitals in Jakarta.Methods This was an observational case series study on full-termand preterm infants who had seizures during hospitalization in CiptoMangunkusumo, Harapan Kita, and Fatmawati Hospitals betweenJanuary-June 2005. Neonatal seizures were defined as seizuresoccurring in the first 28 days of life of a term infant or 44 completedweeks of the infant’s conception age of preterm infant.Results There were 40 neonates who born within the study periodand had seizures. Girls were outnumbered boys. Most neonateswere full-term with birth weight of more than 2500 grams. Analy-ses were done on 38 neonates with epileptic and non-epilepticseizures, while the other two who had mixed clinical manifestationwere not included. Most seizures occurred in the first 3 days of life(23/38). The most common type was focal clonic (12/14) followedby general tonic (11/24) and motor automatism or subtle (10/24).The most common etiology was hypoxic ischemic encephalopa-thy (HIE) (19/38) followed by metabolic disturbances, mainly hy-pocalcaemia (11/38).Conclusion Common types of seizures in neonates were focalclonic, general tonic, and motor automatism (subtle). The mostcommon etiology was HIE followed by metabolic disturbances,mainly hypocalcaemia

Psychological and stabilographic features of healthy persons with different capabilities of performing dual tasks

A dual task study including control of postural balance and counting was conducted on 40 healthy subjects (with an average age of 29.8 ± 2.47 years) along with a psychological examination that included estimation of the working memory, attention, and the time of attention switch. Three types of dual task performance were observed: worsening of either one or both components of the dual task, improvement of one component, and improvement of both components of the dual task. 30% of participants performed a dual task better than isolated tasks. Successful performance in the dual task correlated with the higher volume of spatial and working memory, attention, and a rapid switch of attention. The speed of sway of the center of pressure (CoP) negatively correlated with the speed of attention switch, while the amplitude of the CoP shift in the frontal plane negatively correlated with the volume of spatial and working memory. These data suggest involvement of the cognitive resources in controlling the upright balance and a high degree of motor automatism when the dual tasks are successfully performed. Our experimental paradigm is expected to be helpful in predicting individual performance in situations with high information loads.

Periventricular nodular heterotopia in adult with temporal epilepsy. Forensic implications

Neuronal heterotopias are malformations of neuronal migration, clinically associated with mental retardation, epilepsy and dyslexia. Nodular heterotopias are usually identified in childhood as part of syndromes associating multiple cerebral and systemic malformations. Milder forms may go unrecognized until adulthood when their initial symptoms are usually seizures. We present the case of a 54 years old woman, who is a medical nurse, with temporal lobe epilepsy, with late recognition of her disease. The clinical presentation suggested temporal lobe epilepsy with complex partial seizures, impaired consciousness and motor automatism. Video-EEG has simultaneously recorded the clinical seizure and the ictal paroxysmal discharges. The cause of the temporal epilepsy was proved to be periventricular nodular heterotopia (PVNH).

Cambios en resonancia magnética funcional de la corteza sensitivomotora en pacientes con traumatismo craneoencefálico tras un programa intensivo de rehabilitación

To evaluate the potential reorganization of the sensorimotor cortex in a patient with traumatic brain injury after an intensive motor rehabilitation. A 17-year-old male with severe traumatic brain injury was submitted to functional magnetic resonance imaging (fMRI) analyses of motor control before and after motor rehabilitation. The motor tasks performed during fMRI were finger tapping, ankle plantar flexion, and toe flexion. Prior to treatment, the cerebrally activated areas for the right hand during finger tapping were the primary motor (M1), supplementary motor area (SMA), superior parietal and postcentral areas. For the left hand, the areas were the M1 and the cerebellum. After treatment, the activated areas were the pre and postcentral areas for the right hand and the precentral area for the left hand. For the foot motor-task, the activated areas prior to treatment were the paracentral area for the right foot, and the SMA, paracentral and poscentral areas for the left. After treatment, activation for the right foot was seen in the paracentral area, and activation for the left foot was seen in the paracentral area and SMA. The decrease in the post-treatment activation pattern could be explained as a cortical reorganization, which in the current study was related to motor skill and motor automatism acquired by the patient.

Gertrude Stein (1874–1946) and the nucleus of Darkschewitsch: a discursive commentary

Gertrude Stein is an icon of American literature whose scientific and medical background has become shrouded in obscurity. As an undergraduate at Radcliffe she was strongly influenced by William James and published two papers on motor automatism in the Psychological Review. As a medical student at Johns Hopkins University, her research on the nucleus of Darkschewitsch was quoted in Lewellys F Barker's acclaimed textbook on neuroanatomy; Stein's first book appearance. The background of the Russian neurologist Liverji O Darkschewitsch, little known in the West, is explored particularly in regard to his relationship and collaboration with Sigmund Freud whose letters provide considerable insight. Gertrude Stein failed to graduate with her class of 1901 at Johns Hopkins and soon after departed for an expatriate life in Europe devoted to art and literature.

Dysgraphia in elderly delirious medical inpatients

Dysgraphia is a recognized clinical finding in delirium, but few studies have evaluated handwriting, and results have been inconsistent. In particular, handwritten signatures, which may be a motor automatism, have not been previously evaluated in delirious patients. The aim was to assess abnormalities of signature and spontaneous writing in delirious patients and to investigate their clinical utility in the detection of delirium. Secondary analysis of data was collected from a prospective observational study of acutely ill inpatients 70 years or older. Mini-Mental State Examination, Confusion Assessment Method, Delirium Rating Scale, Activities of Daily Living, and APACHE II were administered to each subject, their signatures were evaluated from the consent form, and their handwriting from the spontaneous sentence written as part of the MMSE. The signatures of patients with delirium were significantly more impaired than those without (Chi-square= 14.749, df=1, p<0.0001). The sensitivity of the signature for delirium as defined by CAM was 0.54, with specificity of 0.88. Handwriting abnormalities of omission (p=0.018), illegibility (p=0.034) and spelling (p=0.035) were significantly more common in delirious patients than others (Chi-square with Fisher's Exact tests. This difference was mainly attributable to the fact that a large number of delirious patients were unable to provide any response to the handwriting questions. An abnormal signature may be an indicator of delirium. People with delirium have handwriting problems, which may be partly caused by cognitive impairment but also by disorders of motor function.

Can homeopaths detect homeopathic medicines by dowsing? A randomized, double-blind, placebo-controlled trial

Dowsing is a method of problem-solving that uses a motor automatism, amplified through a pendulum or similar device. In a homeopathic context, it is used as an aid to prescribing and as a tool to identify miasm or toxin load. A randomized double-blind trial was conducted to determine whether six dowsing homeopaths were able to distinguish between Bryonia in a 12c potency and placebo by use of dowsing alone. The homeopathic medicine Bryonia was correctly identified in 48.1% of bottle pairs (n=156; 95% confidence interval 40.2%, 56.0%; P=0.689). These results, wholly negative, add to doubts whether dowsing in this context can yield objective information.

Stein's Stein: A Tale from "The Aphoristic Theatre"

Among his oblique and eccentric entries into domain was a study of spiritualist practice of trance-induced automatic writing, much to dismay of his more rationalist colleagues. In this light, he initiated a series of psychological experiments in which subject was tested according to varying degrees of fatigue and distraction. One of his subjects was Miss Stein, whom he was to describe as the ideal student. She recounts her own experience: Strange fancies begin to crowd upon her, she feels that silent pen is writing on and on forever. Her record is there, she cannot escape it and group about her begin to assume shape of mocking friends gloating over her imprisoned misery. Bizarre as this might be, it was in any case better than those experiments conducted by one of her classmates, notably less than ideal, who complained to Professor James that consistent lack of response by one of students was falsifying results of his work. When asked who student was, he replied that it was Gertrude Stein. The Harvard professor, true to form, found her lack of response to be perfectly normal. Stein was greatly amused by this incident, remembering that after French Revolution, Saint-Just proposed that in lycees prize for eloquence be given for laconism. Of course, tightlipped New Englanders who were her classmates would certainly understand such a procedure; for her, coming from San Francisco, this was a quite novel approach to things. In any case, in his wisdom, James was much later to ?xplain to his prize student: Never reject anything. Nothing has been proved. Inspired by her mentor, Gertrude Stein was immediately to begin a series of supplementary experiments with her friend, Leon Solomons, results of which were published in Harvard Psychological Review of I896, entitled Normal Motor Automatism. This was Gertrude Stein's first publication. There were only two subjects, herself and Solomons. Her description: This is a very pretty experiment because it is quite easy and results are very satisfactory. The subject reads in a low voice, and preferably something comparatively uninteresting, while

Phenomenology of changed cognitive states in paraphilia

The results of clinicopsychopathological examination of 200 men against whom legal proceedings were instituted for sex offences were presented. Common for them were perceptual disturbances in the form of dere alization, states of "deja vu" and "jamais vu", decrease or complete disappearance of perception stimuli of every modality; disorientation in space and time, own persona lity; disturbance of associative sphere; speech and motor automatism; disturbance of memory in the form of hy permnesia, "dissociation" amnesia etc. 5 kinds of state of disturbance of consciousness were distinguished those resembling "special'' Gurevich states of consciousness, dissociative disturbance .of consciousness in the form of trance, with diencephalic symptomatology prevalence, twilight states, affectively narrowed disturbance of consciousness.

Surgical treatment of partial epilepsy arising from the insular cortex

Despite its documented connections with many limbic structures, the role of the insula in the etiology of partial seizures is poorly understood. Two patients are described in whom lesions of the insula were associated with intractable partial seizures. In the first patient, the seizures involved visceral sensory hallucinations followed by motor automatism. Seizures in the second patient began with somatic sensory hallucinations and then produced visceral motor effects. Both patients were found to have low-grade astrocytomas of the insula. In both instances, resection of the lesion and adjacent insular cortex resulted in a cure of the seizures. These cases are placed within the context of the existing literature on the subject.

Human locomotor adjustments during perturbed walking

The locomotor adjustment induced by step perturbation of human subjects walking on a treadmill was described by a quantitative analysis of the EMG activities of selected trunk and leg muscles and by rotations of leg joints. The role of the proprioceptive input in the EMG reaction was also evaluated. The perturbation was obtained by a rapid and unexpected increase of belt speed. The motor response showed the stereotyped characteristics of a motor automatism and was accomplished without affecting the basic motor pattern of the gait. The EMG adjustment showed short-latency reflex responses (40–60 msec) of muscles acting at the joints more directly affected by the perturbing stimulus. This result supports the hypothesis of a spinal neuronal mechanism involved in the rapid adjustment of gait. The activity of primary spindle afferents seems to play an important role in the production of the faster EMG responses.

Atropine stereotypy as a behavioral trap: a movement subsystem and electroencephalographic analysis.

An analysis was made of the movement subsystems involved in the stereotyped behaviors and electroencephalographic (EEG) activity that appear in rats given the anticholinergic drug atropine sulfate (50-75 mg/kg). One form of stereotypy occurred when the drugged faced the closed end of an alleyway. Instead of scanning briefly and then turning around to face the open end, as undrugged rats do, they typically "trapped", i.e., they continued to engage in rapid, repetitive scanning by the snout up and down or side to side along the surfaces of the closed end for long periods of time. Atropine appears to produce an exaggerated snout thigmotaxis. Accordingly, the behavioral pattern can be manipulated simply by changing the configuration of the environment, which alters sensory input to the snout. Therefore, such stereotyped behavior is not a motor automatism but rather a circular chain of reflexive reactions to surfaces which trap the animal. During rapid shifts in direction of movement in undrugged rats, there were often extremely brief periods of hippocampal theta that could be blocked by atropine. The presence of theta during such shifts might be important for normal behavioral sequencing. This could partially account for the fact that atropine-treated rats failed to change from their initial reactions in the alleyway to more adaptive behaviors. When a low roof was placed over the alleway, head scanning was greatly limited, and immobility in a lying posture rapidly became the more frequent behavior. This immobility was accompanied by a sleeplike (synchronized) neocortical EEG pattern rather than the activated (desynchronized) neocortical pattern that occurs during repetitive thigmotactic scanning. It is hypothesized that scanning stereotypy and activated EEG are maintained through movement-concurrent positive feedback.

Effects of deafening and blindfolding on amphetamine induced stereotypy in the cat

The effects of specific sensory deprivation on the stereotypic behavior produced by amphetamine was examined in cats with normal hearing and in experimentally deafened animals. Following amphetamine, deaf animals demonstrated eye and head movements which were qualitatively similar to stereotyped behavior in animals with normal hearing. Blindfolding dramatically decreased amphetamine induced head movement stereotypy in cats with normal hearing and in deafened animals. It is concluded that stereotypy induced by threshold doses of amphetamine in the cat is not due to activation of a pure motor automatism but represents release or facilitation of a centrally patterned exploratory program which is maintained by sensory feedback.

Human Personality and its Survival of Bodily Death

IN introducing this book to what must be regarded for the most part as a hostile audience, I would claim for it that it is a record of the life-work of a pertinacious and industrious student, in a region beyond the borderland of present orthodox science; and would explain that it has for its object the better comprehension and coordination of a multitude of human faculties, some of them recognised as real though obscure, others not yet generally recognised as existing. The phenomena of sleep, of genius, of multiple personality, of hysteria, of hypnotism, of hyperaesthesia, and of trance, are among those generally recognised by medical specialists and practically treated; though, in truth, most of them seem to be regarded chiefly or solely as pathological curiosities. The phenomena of sensory and motor automatism, of telepathy, and of clairvoyance, are not among the human faculties yet generally recognised. By long study Myers was able to accept them all, in various degrees, and he discerned a thread of connection running through them, so that he felt it possible gradually to design a comprehensive scheme which should include them all,—a building, as it were, in the composition of which each constituent filled its appointed place, so that no part was left forlorn and unsupported by adjacent materials, and so that the eye of science subsequently glancing over it might be willing to recognise the possibility and appropriateness of structures which when isolated had seemed strange and fantastic and incredible. Human Personality and its Survival of Bodily Death. By Frederic W. H. Myers. In two volumes. Vol. i. pp. xlvi + 700; vol. ii. pp. xx + 660, including elaborate index. (London: Longmans, Green and Co., 1903.) Price 2l. 2s. net.

Cultivated motor automatism; a study of character in its relation to attention.

Cultivated motor automatism; a study of character in its relation to attention.