BACKGROUND AND SIGNIFICANCE: Oro-facial dyskinesia are stereotyped movements, consisting of smacking and pursing of the lips, lateral deviation and protrusion of the tongue, and occasionally lateral deviation and protrusion of the jaw. Oro-facial dyskinesia rarely occur with Huntington's disease, acquired hepatocerebral degeneration, or other choreatic disorders. Spontaneous oro-facial dyskinesias occur in the elderly and had been said to result from edentulousness. It is important to clarify the pathophysiology of senile oro-facial dyskinesia for the prevention and proper treatment. I have had a doubt on the current concept that edentulism is the main etiologic factor of senile oro-facial dyskinesia. Through the clinical experience, I have had an impression that edentulism might not be the primary underlying cause of senile oro-facial dyskinesia. CASE AND METHODS: I have recently experienced four cases of senile oro-facial dyskinesia. There were no history of being exposed to neuroleptics. I tried to clarify the role of edentulism in senile oro-facial dyskinesia through clinical observations, electrophysiologic studies, and pharmacologic trials. CONCLUSION: Two patients said that wearing of denture might be responsible for the development of oro-facial dyskinesia. EMG studies with some procedures showed somewhat consistent results between two patients who were tested. EMG activities were increased by wearing of denture and decreased by touching on the perioral area with examiner's hand. Furthermore two patients who were medicated with dopamine receptor blocker showed marked clinical improvements. These results suggested that senile degenerative changes of central nervous systems might be the primary cause of senile oro-facial dyskinesia.
Aged ; Antipsychotic Agents ; Central Nervous System ; Chorea ; Dentures ; Dyskinesias* ; Hand ; Hepatolenticular Degeneration ; Humans ; Huntington Disease ; Jaw ; Lip ; Receptors, Dopamine ; Tongue

Ataxia is one of the most serious neurological symptoms in elderly The clarification of the related anatomical structures are necessary for the understanding of pathophysiologic mechanisms of ataxia. We have recently experienced a case of thalamic infarct in the territory of thalamogeniculate artery. The localization of lesion was based on CT and MR imaging. At first time, right hemiparesis and ataxia were prominent. Hemiparesis was transient but ataxia had been persistent for several months. It has been suspected that thalamus could be the part of neural circuits in balancing. Our case support this suggestion clinically. Based on clinical observations, a plausible extrapolation can be made to thalamic ataxia. It maybe related with dysfunction of dentatorubrothalamic and corticopontine pathway. Thus our case led us to conclude that thalamus could be engaged in balance control of human body.
Aged* ; Arteries ; Ataxia* ; Human Body ; Humans ; Magnetic Resonance Imaging ; Paresis ; Thalamus

SUNCT syndrome is a headache syndrome characterized by short-lasting(usually 15-120 sec), unilateral head paroxysms localized in the peri-ocular area, accompanied by conjunctival injection, lacrimation, nasal stuffiness, rhinorrhea, and subclinical forehead sweating, all on the symptomatic side. We experienced a new SUNCT syndrome case. 57-year-old patient had been suffering from right orbital pain attacks since he was 54. The pain, of an ""electrical"" or ""burning"" character, was moderate to severe in intensity. Single attacks lasted for 30-60 seconds each and were accompanied by prominent ipsilateral conjunctival injection and tearing as well as slight rhinorrhea. His symptoms were relieved by carbamazepine. Carbamazepine treatment seemingly brought about marked decrease in the frequency and severity of attacks. This case may be the first reported SUNCT syndrome case in Korea. Furthermore this case may shed light on the nosologic point view of SUNCT syndrome. The suspected pathophysiology of trigeminal neuralgia is expansion of the low threshold portions of the receptive fields owing to loss of surround inhibition. Carbamazepine facilitate afferent inhibition and depress excitatory transmission in the trigeminal nucleus. So the effectiveness to carbamazepine has been considered as one of diagnostic criteria of trigeminal neuralgia. Through the survey of previous reported cases including our case, it is suspected that SUNCT syndrome be closer to trigeminal neuralgia rather than cluster headache. We discussed several viewpoints for the nosologic aspect of SUNCT syndrome in this report.
Carbamazepine* ; Cluster Headache ; Forehead ; Head ; Headache Disorders ; Humans ; Korea ; Middle Aged ; Orbit ; SUNCT Syndrome* ; Sweat ; Sweating ; Trigeminal Neuralgia ; Trigeminal Nuclei

BACKGROUND AND PURPOSE: Dystonia is not understood and its pathophysiology is uncertain. The fundamental motor abnormality is an abnormality of muscle command signals, in such that the wrong agonists may be activated for too long, there is abnormal co-contraction of agonists and antagonist, and there is excessive and misdirected action of synergists and postural fixators. The reciprocal inhibition of H reflex has been studied for the evaluation of pathophysiology in tonus abnormalities. Lelli et al.(1991) found the similarities in reciprocal inhibition studies between dystonia and Parkinson's disease. He suggested that abnormalities of reciprocal inhibition appear to be a physiologic overlap between two disorders. There is, however, controversy about the results of reciprocal inhibition studies in both disorders. The cutaneous reflex study is another valuable test for the evaluation of the pathophysiology in tonus abnormalities. In an attempt to clarify the pathophysiologic mechanisms of both disorders, we performed cutaneous reflex tests and compared the findings of both disorders with those of the control group. METHODS: 50 normal persons as a control group, 10 patients with dystonia, and 10 patients with Parkinson's disease participated in this study. Cutaneous reflex responses were recorded in the first dorsal interosseous muscle following electrical stimulation of the digital nerves of the index finger. RESULTS: Each mean amplitude of I1 of cutaneous reflex responses in patients with dystonia and in patients with Parkinson's disease is significantly decreased as compared with those of the control group. CONCLUSION: The results from this study lead us to conclude that both dystonia and Parkinson's disease might have abnormalities of inhibitory supraspinal influences on spinal mechanisms.
Dystonia* ; Electric Stimulation ; Fingers ; H-Reflex ; Humans ; Parkinson Disease* ; Reflex*

Post-traumatic encephalopathy is characterized by the combination of upper motor neurons, basal ganglia, cerebellar, and psychiatric disturbances. A "striatal" variant, with predominant parkinsonism, is uncommon and may be difficult to distinguish from idiopathic Parkinson's disease. A 38-year-old man, who had history of head injuries associated with brief losses of consciousness, developed a resting tremor and rigidity of the left lower extremity. Upon admission, he showed masked face and bradykinesia without dementia or other neurologic abnormalities. Brain MRI and SPECT studies showed no abnormalities. A BAEP study revealed an abnormal prolongation of inter-peak latencies between wave III and V.
Adult ; Basal Ganglia ; Brain ; Brain Injuries ; Consciousness ; Craniocerebral Trauma* ; Dementia ; Head* ; Humans ; Hypokinesia ; Lower Extremity ; Magnetic Resonance Imaging ; Masks ; Motor Neurons ; Parkinson Disease ; Parkinsonian Disorders* ; Tomography, Emission-Computed, Single-Photon ; Tremor

Post-traumatic encephalopathy is characterized by the combination of upper motor neurons, basal ganglia, cerebellar, and psychiatric disturbances. A "striatal" variant, with predominant parkinsonism, is uncommon and may be difficult to distinguish from idiopathic Parkinson's disease. A 38-year-old man, who had history of head injuries associated with brief losses of consciousness, developed a resting tremor and rigidity of the left lower extremity. Upon admission, he showed masked face and bradykinesia without dementia or other neurologic abnormalities. Brain MRI and SPECT studies showed no abnormalities. A BAEP study revealed an abnormal prolongation of inter-peak latencies between wave III and V.
Adult ; Basal Ganglia ; Brain ; Brain Injuries ; Consciousness ; Craniocerebral Trauma* ; Dementia ; Head* ; Humans ; Hypokinesia ; Lower Extremity ; Magnetic Resonance Imaging ; Masks ; Motor Neurons ; Parkinson Disease ; Parkinsonian Disorders* ; Tomography, Emission-Computed, Single-Photon ; Tremor

CHAP syndorme consists of choreoathetosis, orofacial dyskinesia, affective change, hypotonia, and pseudobulbar signs. CHAP syndrome was firstly des- cribed with onset 1 to 7 days after induced profound hypothermia and complete circulatory arrest for con- genital heart lesion repair. Since then, only a few cases have been reported until now. Case We report a patient who demonstrated all of the elements of the CHAP syndrome in severe cachexic state. Brain SPECT(99mTC-ECD) shows decreased perfusion in left fronto-parieto-temporal lobe. Comments The pathogenesis of CHAP syndrome is obscure. However our case led us to suggest that more essential component pre- disposing to the CHAP syndrome is hypoxia rather than hypothermia. Furthermore, it seems to be related with circulatory insufficiency in extrapyramidal nervous systems.
Anoxia ; Brain ; Heart ; Humans ; Hypothermia ; Movement Disorders ; Muscle Hypotonia ; Nervous System ; Perfusion

BACKGROUND: Continuous lingual myoclonus is an exceptional entity usually described in association with similar palatal, ocular, facial, diaphragmatic, or shoulders movements. Only a few cases of isolated lingual myoclonus have been reported until now, and the pathophysiology of lingual myoclonus is unclear. CASE: We have recently experienced a case of lingual myoclonus. A 27-year-old female patient was noted to have continuous involuntary contractions involving exclusively the tongue. Electromyographic study showed low frequency(2 Hz), more or less rhythmic bursts from right genioglossus muscle. There were no abnormalities in brain evoked potentials and brain MRI. Lingual myoclonus showed good response to valproic acid and botulinum toxin. COMMENT: Embryologically soft palate and tongue are different. Soft palate is both somatic and branchial origin, but tongue is somatic origin. And lingual myoclonus is more irregular than palatal myoclonus. Therefore, it is suspected that the pathophysiologic mechanisms of lingual myoclonus are somewhat different from those of palatal myoclonus.
Adult ; Botulinum Toxins ; Brain ; Evoked Potentials ; Female ; Humans ; Magnetic Resonance Imaging ; Myoclonus* ; Palate, Soft ; Shoulder ; Tongue ; Valproic Acid

BACKGROUND AND OBJECTIVE: Transient global amnesia(TGA) is a neurological syndrome affecting preferentially middle-aged or elderly people and characterized by sudden onset of transient impairment of antegrade amnesia with variably retrograde amnesia. TGA was defined over 30 years ago, but the etiology remain unclear. Several mechanisms have been proposed, including arterial thromboembolic ischemic attacks in both posterior cerebral artery territories, epilepsy, and migraine. Although many studies have reported TGA, only a few reported cerebral perfusion studies using SPECT because of the brief duration of the episode. Technetium-99m-ethyl cysteinate dimmer(ECD) is superior to sensitivity of lesion detection and lesion to normal contrast, probably due to lower back diffusion from the brain to the blood and its excellent radiochemical stability. So we evaluate pathophysiologic mechanism of TGA by using 99mTC-ECD SPECT with or without acetazolamide(ACZ) stress. METHODS: We evaluated six patients with transient global amnesia, four women and two men, age ranges 56 to 78 years, mean 62.2 years. Measurements of CBF and vascular reserve using 99mTC-ECD with or without ACD stress were performed during or after TGA episode. RESULTS: One patient, who was evaluated in TGA episode showed that regional cerebral blood flow was decreased in both anteroinferior frontal, both temporal, right thalamus, both inferior parietal and left parietal region with impaired vascular reserve in left inferior temporal and right thalamus. Others, who were evaluated more than 6days in TGA episode showed that regional cerebral blood flow was decreased in left temporal (4), both temporal region (1), left thalamus (2) and both basal ganglia (1) with preserved vascular reserve except one, showed impaired vascular reserve in left thalamus. CONCLUSION: This result show that severe hypoperfusion of bilateral temporal region and impaired vascular reserve in left temporal region in TGA episode. Other patients who were recovered from TGA showed hypoperfusion of left temporal region and preserved vascular reserve. 99mTc-ECD with acetazolamide SEPCT that sensitively detects localized impaired cerebraovascular reserve should help elucidate these processes. But further study with more cases is necessary for evaluation of pathophysiology of TGA.
Acetazolamide ; Aged ; Amnesia ; Amnesia, Retrograde ; Amnesia, Transient Global* ; Basal Ganglia ; Brain ; Diffusion ; Epilepsy ; Female ; Humans ; Male ; Migraine Disorders ; Perfusion ; Posterior Cerebral Artery ; Rabeprazole ; Technetium* ; Thalamus ; Tomography, Emission-Computed, Single-Photon*

Ocular flutter is a rare, horizontal eye movement disorder characterized by rapid saccadic oscillations. Previous reports have presented a relationship between ocular flutter syndrome and many conditions/diseases. However, it is surprising that there have been few reported cases in which there has been a clear anatomically localized lesion linked to ocular flutter. So the pathophysiological basis for ocular flutter syndrome is not understood yet. We have recently experienced a case of ocular flutter syndrome where the ocular flutter was associated with dysmetria, dysdiadochokinesia, kinetic predominant tremor, and ataxia. The prodromal symptoms, self-limited illness course and cerebrospinal fluid (CSF) pleocytosis of our case suggest an infectious aetiology for this syndrome. Although most of the sequelae of infectious diseases represent more diffuse involvement of the nervous system, the predominant cerebellar symptoms and signs associated with ocular flutter imply that the single responsible lesion of the present case might have been related to the cerebellum-associated neural circuits. Brain single photon emission computed tomography (SPECT) in the present case showed perfusion decrease in the left thalamus. The present case suggests that the cerebellar output circuit (dorsal vermis-fastigial nucleus-vestibular nucleus-thalamus-cortex) might be related to the occurrence of ocular flutter. The description of this case may improve the understanding of the pathological mechanisms of ocular flutter syndrome.
Ataxia ; Brain ; Cerebellar Ataxia ; Cerebrospinal Fluid ; Communicable Diseases ; Leukocytosis ; Nervous System ; Ocular Motility Disorders ; Perfusion ; Prodromal Symptoms ; Thalamus ; Tomography, Emission-Computed, Single-Photon ; Tremor