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    Home > Active Ingredient News > Study of Nervous System > Involuntarily bite tongue, dance-like movements, what diseasedos do you consider?

    Involuntarily bite tongue, dance-like movements, what diseasedos do you consider?

    • Last Update: 2020-06-16
    • Source: Internet
    • Author: User
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    Case Review The patient, a 38-year-old man, was admitted to hospital on June 8, 2020 due to "involuntary shaking of the head, torso and limbs for more than 9 months."Current medical history: 9 months ago due to emotional stimulation after the appearance of involuntary tongue bite and cheek mucosa, initially not pay attention to, the symptoms gradually worsened, in recent months began to appear head, torso and limbs involuntarily shake, emotional agitation or tension when aggravated, calm state, before falling asleep quiet can be reduced, after falling asleep can be reduced, after falling asleep, with a crowded eyebrows, mouth, tongue spitting, face shaking, jaw, speech clumsiness, sometimes clumsy, and sometimes speechUp and down the stairs or ground is not usual, slightly do not pay attention to easy to fall, no limb numbness, no difficulty swallowing, no eating cough, no intelligent hypothermia, no sound and visual hallucinations, unconscious disorder, to the nearby community health service center, to give 'methylcobalamines' nutritional nerve therapy, symptoms no significant improvement, the course of the disease, the patient denied chill fever, denied chest pain, denied heart palpitations, denied diarrhea, vomiting, eatingPast history: physical fitness, denied the history of drug use, the character of the child is more lively and active, denied tobacco and alcohol and other hobbies, unmarried infertile, complained that his father has mental disordersAdmission: T:36.3C, P:98 times/min, R:18/min, BP:115/69mmHg, Mind clear, mental, no neck blood vesselabnormal throbbing or murmuring; fluent speech, clear spitting, eye movement, eye tremor (-), double Side eye cracking and other large, two-sided pupils and other large iso-circular, diameter of about 3.0mm, normal reflection of light, two-sided nasal lip groove symmetry, tongue-in-the-middle, tongue-free atrophy, dangling center, pharynx reflection (plus), shrug, neck side symmetry strong, rough double-ear hearing symmetryBuch, Krzyscopic (-), limbs, torso and head involuntary movement, no muscle beam tremor, double lower limb tendon reflection weakened, double upper limb tendon reflection (), limb muscle tension slightly higher, limb near end muscle strength, far end muscle force are 5 levels; fatigue test (-), two-sided pathological signs (-), two-sided deep-light feeling normalTwo-sided finger-nose test, knee-to-knee test (-), closed eye difficult to sign up test (-)After admission to improve the relevant examination: blood routine, electrolyte, clotting function, hepatitis B, hepatitis C, syphilis, HIV, methyl polysix, homocysteine, folic acid, vitamin B12, blood sink, anti-O, rheumatoid factor, C-reactive protein are in the normal rangeBiochemical full set TG, TCH, LDL-C, HDL-C are all in the normal range, creatinine 72.4 smol/L, urea 5.84mmol/L, glucose 4.96mmol/L, glutauine transaminase 22.3U/L, creatine kinase 903.1U/L (normal value 24-195 U/L), lactic acid dehydrogenase 270.4U/L (normal 80-245 U/L), LDL 2.05mmol/LCardiomyase spectrum: creatine kinase 891.7U/L, a-hydroxybutyinin dehydrogenase 221.5U/L, lactic acid dehydrogenase 269.1U/L, CK-MB (active) 28.1U/L (normal 0-25 U/L)Head and seahorse nuclear magnetism: 1The cranial brain MRI flat-sweep brain did not see significant abnormalities in substance; 2Haima flat sweep did not see obvious abnormalities, please combine clinicalEEG: Both sides are more scattered in and burst spiky sharp waves, sharp slow waves, ripplesThe ophthalmology consultation did not see the "K-F ring"Copper-blue protein is normal Peripheral blood smears, echidna cells accounted for 16%, etifying cells irregular contours, cells are retracted, echidnas are less, distribution is different, the end of the echidna is often spherical Diagnosis: neuroenablastation (neuroacanthocytosis, NA) Figure 1 Peripheral blood smear examination Discussion Neuroenablastation is a rare genetic disease, a progressive neurodegenerative disease that mainly affects the substrate nerve section, accompanied by an increase in echidna in peripheral blood, first reported by Levine and Critchley in 1960 With movement disorders (dance disorders, convulsive disorder, jaw movement disorders, Parkinson's syndrome, etc.), personality changes, progressive intelligence decline, peripheral neuropathy and the increase of peripheral blood ratchet red blood cells (-3%) For typical clinical manifestations Most of the disease is 20-30 years old, most of them with body dance-like movements or involuntary movement of the lips and tongue as the first symptoms In recent years, it has been made clear that the genes of CHAC are located at 9q12, and its genetic form is generally consistent with autosomal recessive genetics, and that the father has mental disorders, with the exception of further peripheral blood smears The characteristics of the patient's case: middle-aged male, chronic onsthep, aggravation Involuntary bite of the tongue and the cheek mucous membrane, for the mouth muscle dystonia Head, torso and limbs shaking involuntarily, accompanied by frowning, mouthing, spitting tongues, posing as faces, shaking jaws, clumsy speech, sometimes speech and stop, for the manifestation of dance disorders Peripheral blood smears, 16% of echidna In accordance with the diagnosis of neuroesolucine hypertrophy The two sides of the patient's eEG map are more scattered in and the burst of sharp spiky, sharp slow waves, ripples, no eclampsia, but indicated that there is brain damage Hardie et al class divides NA into type 3: Bassen-Kornz-weig syndrome The basic performance is erythropoietin, beta-lipoprotein deficiency, poor fat absorption, co-relief disorders, etc., can accompany muscle atrophy, bow foot and so on McLeod syndrome The main manifestations of various movement disorders, but often have reflection loss, myopathy, cardiomyopathy, creatine kinase activity increased and long-lasting hemolytic state, red blood cells show Kell antigen and XK antigen antigen antigen significantly weakened or disappeared Levin-Critch-ley syndrome The performance of various motor disorders, similar to Mcleod syndrome, but the patient's red blood cell surface Kell antigen and XK antigen performance is normal, serum lipoprotein levels are also in the normal range The patients TG, TCH, LDL-C, HDL-C are all in the normal range, do not support Bassen-Kornzweig syndrome; Except for the further examination of Levin-Critch-ley syndrome Differential diagnosis of Huntington disease Its clinical manifestations are similar to neuroesolucine aneurysm, but the former has no peripheral blood ratchet red blood cell growth Twitching 'Tourette syndrome) More common in children, symptoms are more confined to the face, even can be tired of the upper limbs, the main performance of irregular pumping, no dance symptoms, no ratchet red blood cell increase, the disease is easy for adults and NA identification Hepatic bean-like nuclei Patients young, limb dance-like movements or involuntary movement of the lips and tongues, should be distinguished from the liver bean-like nuclear degeneration, but the patient's liver function is normal, ophthalmology consultation did not see "K-F ring." Copper-blue protein is normal, except Small dance disease Multi-coyote is found in girls between 5 and 15 years of age, the performance is similar to NA, no peripheral blood ratchet red blood cell increase, the disease is middle-aged people, except Treatment So far, the TREATMENT of NA non-specific drug treatment, generally believed that Antan, brominated, chloroneoxe, anti-5-HT preparations, etc., are ineffective for involuntary movement, l-dopa formulations and fluorool efficacy evaluation is not the same It is also thought that large doses of vitamin E can be tried to change the fluidity of red blood cell membranes, while the use of fluorool and other control dance symptoms Source: Network
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