Creutzfeldt-Jakob disease (CJD) is a rare dementing illness that usually affects older adults and is characterized by a rapidly progressive dementia, ataxia, and myoclonus. The disease is due to an unusual transmissible protein called a prion. The diagnosis is often suspected on the basis of electroencephalographic (EEG) and clinical findings. Our case was 62-year-old woman, who presented acute onset rapid progressive dementia, myoclonus, heightened startle reaction, extrapyramidal symptoms, and died about 2.5 months after onset of disease. We diagnosed this case as CJD with typical EEG pattern and clinical features.
Adult ; Ataxia ; Creutzfeldt-Jakob Syndrome* ; Dementia* ; Diagnosis ; Electroencephalography ; Female ; Humans ; Middle Aged ; Myoclonus ; Startle Reaction

Animals ; Reflex, Startle ; Models, Animal ; Acoustics

Prepulse inhibition (PPI) is suppression of the startle reflex when an intense startling stimulus is preceded by a weaker sensory stimulus (the prepulse). It is an operational measurement of sensorimotor gating mechanism to help human adapt to complex environment. This weak prepulse protect central cognitive processing by damping the effect of intense stimuli. Autistics cannot select out behaviorally important information from a lot of irrelevant resources and reflect abnormal gating mechanism and attentional abnormalities. Previous studies have not made agreement on whether autistic patients demonstrated deficits in PPI, because the results depend on age, sex, severity of the disease as well as the experimental parameters used. Moreover, these studies have not covered whether autistics have suffered deficits in higher-order processing. In this review, the "top-down" modulation of selective attention and subjective emotion are introduced into the PPI experiment. We also introduce fear conditioning and perceived spatial separation paradigm to further explore the interaction between autistic cognitive process and gating mechanism.
Attention ; Autistic Disorder ; Fear ; Humans ; Prepulse Inhibition ; Reflex, Startle

Early diagnosis of persistent abnormal reflexes may be of great significance to a more effective functioning of the cerebral palsied child. It is important to know the normal and abnormal reflex responses and their effect upon motor development for providing a basis for evaluation in the diagnosis and treatment of the cerebral palsied child. We studied 28 cerebral palsied children, between 1 to 12 years old, who were treated at Sam Yook Childrens Rehabilitation Center from May 1975 to December 1977. The pathologic reflexes were checked, and the relationship between pathologic reflexes and walking was compared. The following results were obtained. 1. The following were the most important pathologic reflexes in non-walking fgroup: a. Positive supporting reaction b. Protective extensor thrust c. Moro reflex 2. If they are present, the prognosis for walking ambulation was bad and surgery will not improve for the chances of walking.
Child ; Diagnosis ; Early Diagnosis ; Humans ; Prognosis ; Reflex ; Reflex, Abnormal ; Reflex, Startle ; Rehabilitation Centers ; Walking

The original article was reported by Creutzfeld in 1920, and Jakob in 1921 respectively. CJD is an encephalopathy with worldwide distribution caused by an unidentified unconventional virus. The principal clinical features of CJD are dementia, upper and in some instances, lower motor neuron disorders; basal ganglionic and cerebellar dysfunction; and myoclonus including a heightened startle reaction. The disease is rapidly progressive, leading in several months to stupor, coma, and death. Pathologic changes are widespread, involving the cerebral cortex, basal ganglia, cerebellum, pyramidal tracts, and lower motor neruons of the brain stem and/or spinal cord. This case was 51 years old Korean male, who presented progressive dementia, myoclonus, heightened startle reaction, pyramidal, extrapyramidal symptoms, decerebrated rigidity, and died about 4 months after onset. We diagnosed this case as CJD with typical serial EEG pattern and clinical feature.
Basal Ganglia ; Brain Stem ; Cerebellar Diseases ; Cerebellum ; Cerebral Cortex ; Coma ; Creutzfeldt-Jakob Syndrome* ; Dementia ; Electroencephalography ; Ganglion Cysts ; Humans ; Male ; Middle Aged ; Motor Neurons ; Myoclonus ; Pyramidal Tracts ; Spinal Cord ; Startle Reaction ; Stupor

Gap prepulse inhibition of acoustic startle (GPIAS) method has been used effectively for the objective assessment of tinnitus in animals. Among two types of enclosures for the GPIAS, the unconstrained type carries less risk of animal death due to the absence of binding stress in the enclosure, and lack of need for alteration to animal size variation as it grows. However, animals' voluntary movements, which have no relation to the startles evoked by acoustic stimuli, are problematic, as they cannot be excluded in the case of the unconstrained enclosure based GPIAS measurement system. In order to discount voluntary movements which are not associated with external acoustic stimuli, we propose the conditional random interstimulus interval (CR ISI) method for unconstrained enclosure based GPIAS measurement. With the proposed ISI method, the unconstrained enclosure based acoustic startle response measurement system has been implemented in this paper. As a result, the effectiveness of the proposed CR ISI method has been verified and compared with those of conventional ISI methods through animal experiments using SD-rats. The experimental results showed that abnormal startle responses and invalid GPIAS values caused by motion were prevented when our proposed CR ISI method was applied to our implemented system. It was also verified that our proposed CR ISI method is advantageous in reducing the total experimental time for acquiring normal startle responses and valid GPIAS values, compared to conventional ISI methods, since our proposed CR ISI can begin the acoustic stimulation only when the animal gets stable and motionless.
Acoustic Stimulation ; Acoustics ; Animal Experimentation ; Animals ; Integrin alpha2 ; Methods ; Prepulse Inhibition ; Reflex, Startle ; Tinnitus

The relative incidence of cerebral palsy has increased recently over that of poliomyelitis, due to the improvement in the social, medical, and economic status of our country. It is important to check the pathologic reflexes for diagnosis, treatment and prognosis of cerebral palsy. We studied 84 cerebral palsied patients, between 1 to 13 years old, who were treated at our Crippled Childrens Rehabilitation Center. The motor age and pathologic reflexes were checked, and pathologic reflexes were checked, and the relationship between pathologic reflexes and walking was compared. The following results were obtained. 1) With increasing of chronological age, there was a tendericy to increasing of motor age, but the mean motor age did not increase after 8 years in chronological age, and no more than to about 20 months. 2) With increasing of motor age, a tendency to decreasing of frequency of pathologic reflexes was shown. 3) The followings were the most important pathologic reflexes in non walking group: a) positive supporting reaction b) supine lying c) prone lying d) Moro reflex e) asymetrical tonic neck f) protective extensor thrust.
Cerebral Palsy ; Child ; Clinical Study ; Deception ; Diagnosis ; Humans ; Incidence ; Neck ; Poliomyelitis ; Prognosis ; Reflex ; Reflex, Startle ; Rehabilitation Centers ; Walking

AIMTo inquire a new credible animal model in studies of startle conditioned reflex.

METHODSThrough the trials of combining a conditioned stimulus (a tone) with an unconditioned stimulus(a foot shock), the startle responses were established in animals by conditioned stimulus. Arterial blood pressure were measured before and after blocking basolateral and lateral amygdala with lidocaine.

RESULTSThe blood pressure was increased by the conditioned stimulus after four days training. When the basolateral amygdala was blocked by lidocaine, the blood pressure was not increased by the conditioned stimulus.

CONCLUSIONThe animal model used and verified in this experiment is a new credible chronic animal model in startle conditioned reflex by measuring arterial blood pressure.

Animals ; Arteries ; physiology ; Blood Pressure ; Conditioning, Classical ; physiology ; Female ; Male ; Models, Animal ; Rats ; Rats, Wistar ; Reflex, Startle ; physiology

Infants with Sandhoff disease typically appear normal until 3–6 months of age. As the disease progresses, they present with symptoms such as loss of motor skills, exaggerated startle response to loud noise, seizures, visual loss, and paralysis. We encountered a rare case of a 22-month-old girl with Sandhoff disease characterized by progressive motor weakness and dysphagia, who initially showed signs of aspiration at 20 months of age. The major problems related to dysphagia were oromotor dysfunction and abnormal feeding posture. Within 3 months of identification of difficulty in swallowing, the patient showed a significant decrease in food intake, with rapid deterioration of nutritional status. We report our case with a review of the literature.
Deglutition Disorders ; Deglutition* ; Eating ; Female ; Humans ; Infant ; Motor Skills ; Noise ; Nutritional Status ; Paralysis ; Posture ; Reflex, Startle ; Sandhoff Disease* ; Seizures

Prepulse inhibition (PPI) is considered to be one of the most promising neurophysiological indexes for translational research in psychiatry. Impairment of PPI has been reported in several psychiatric diseases, particularly schizophrenia, where PPI is considered a candidate intermediate phenotype (endophenotype) of the disease. Recent findings from a variety of research areas have provided important evidence regarding PPI impairment. Human brain imaging studies have demonstrated the involvement of the striatum, hippocampus, thalamus and frontal and parietal cortical regions in PPI. In addition, several genetic polymorphisms, including variations in the genes coding for Catechol O-methyltransferase, Neuregulin 1, nuclear factor kappa-B subunit 3 and serotonin-2A receptor were related to PPI; and these findings support PPI as a polygenetic trait that involves several neurotransmitter pathways. Early psychosis studies suggest that PPI disruption is present before the onset of psychosis. Also, discrepancy of PPI impairment between children and adults can be found in other psychiatric diseases, such as autistic spectrum disorders and posttraumatic stress disorder, and comprehensive investigation of startle response might contribute to understand the impairment of the neural circuitry in psychiatric diseases. Finally, recent studies with both Asian and Caucasian subjects indicate that patients with schizophrenia exhibit impaired PPI, and impaired sensorimotor gating might be a global common psychophysiological feature of schizophrenia. In conclusion, studies of PPI have successfully contributed to a better understanding of the fundamental neural mechanisms underlying sensorimotor gating and will certainly be most valuable in devising future approaches that aim to investigate the complex pathogenesis of psychiatric diseases.
Adult ; Asian Continental Ancestry Group ; Catechol O-Methyltransferase ; Catechols ; Child ; Clinical Coding ; Endophenotypes ; Hippocampus ; Humans ; Mental Disorders ; Neuregulin-1 ; Neuroimaging ; Neurotransmitter Agents ; Phenotype ; Polymorphism, Genetic ; Psychophysiology ; Psychotic Disorders ; Schizophrenia ; Sensory Gating ; Startle Reaction ; Stress Disorders, Post-Traumatic ; Thalamus ; Translational Medical Research