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Late-onset Hallervorden-Spatz disease: a report of autopsy report


; ReporlofacaseofHallervorden-SpalzdiseaseAca5cofHalte ~ vofdenSpatzdiseasewithautopsy {i, adir ~ gsina68 a year-oldmanispresent-ed. Thepatientdiedafter5yearsofprogressiverigldity, maskface, shufflinggaitandtremor. NOfaroilyhistorywasavailable, Atautopsy, therewaslossofneuronswithgliosisintheglobuspallidusandaxonalspheroidswerefoundinthesameareas. Thisisthefirstcas0fI] allervmdenS ~ alzdiseasewithautopsyringsreportedinChina. KeyWords: Hallervorden-SpatzdiseaseGlobuspallidusIronpigmentsSpheroidsDiagnosis, diH sculpture, the performance of specific nerve palsy Neurology, Li Guoping Ren Cao Xian Long from a r ---- __ --- recently treated two cases of a specific clinical manifestations in patients with oculomotor palsy, reported as follows. Example 1 female, 58 years old, suddenly felt dizzy activity, depending on the material double image, the next morning after getting the official language is unclear, the right hand is weak. A day later the right ptosis, left crooked mouth with right hemiplegia. Diabetes for 5 years. Without hypertension, coronary heart disease. I live in families two days later, when the mind indifferent, left upper eyelid completely down, left oblique and inward under the eye outside, up, down, then move can not be left mydriasis 4mm, light reaction disappears, the right upper eyelid drooping slightly, limited mobility in all directions right eye, right central facial paralysis of tongue, base right limb paralysis, low muscle tone, painted light sensory barrier, reflecting lower limbs Kin, right irresolute gf out the pathological can be no meningeal sign Kuai shock . Glucose 422mg, urine volumes. Brain CT showed the left side of the brain near the midline in the sheet density shadow. DSA no carotid and vertebral abnormalities were a hospital diagnosis of basilar artery: ④ vertebral basilar artery thrombosis of a sugar feces ② Case 2 male, 10 years old, after a cold, dizziness, difficulty swallowing and drinking cough. After a few months depending on the material double vision, weakness in January after the elevation of left upper eyelid, the right crooked mouth, and difficulty swallowing after aggravating the left ptosis, intermittent roar of the left ear hearing loss, hoarseness and hormone treatment after the disease was 10 days improved after 4 months admitted to our department. Lee was kind of clear language is normal vision, left ptosis, left eye, the direction of movement was significantly restricted Wei, double the normal size of the pupil Bodhisattva round light response, and left facial cancer feel a bit weak, the right mouth slightly crooked, the rest of the nervous system was normal. Head MRl: left parasellar meet T-plus vote lead of a low signal area of ​​about 25x20ram, prompt action amidine tumor. DSA: left common carotid and internal moving less 777 amidine c. Section giant aneurysm diameter 30ram. Wenquan internal carotid artery aneurysm of left facial pain after restoration plug, numbness of the mouth bilaterally symmetrical, but no obvious signs of nerve damage to improve. Discussion: down 1 weber syndrome and there on the back nerve damage in the brain left brain CT showed low density near the midline, can be sure that the reverse nerve damage is caused by lesions in the brain. The back shows the movement of intramedullary lesions of ventral nerve palsy due to also enable medium and large dilated pupils and loss of brain nerve lesions of abdominal pay back a sense of changes in the brain was covered fiber or nerve lesions affected the upper edge of the ridge Wei Ai ~ Time may also lead to mydriasis. Down the possibility of the latter for large. Back 2 the course of cranial nerve injury and there have been Ⅲ Ⅳ V Ⅵ Ⅷ turn Ⅸ X, etc., before a cold illness history, the hormone significantly improved medical treatments, people at the hospital we have considered the sheath off the brain stem-type disease. Investigation by MRI and was diagnosed with DSA orange c. Giant aneurysms in this segment fell nerve damage as a result of extramedullary disease, and nerve damage extramedullary Cao hole dilated when the earlier and often more significant and timely medical treatment after the illness in this case, to my wrist and then Medical multiple inspections by all levels of extraocular muscle paralysis although very significant, but always to see the pupil Migration of King great. This shows that the severe oculomotor palsy caused by extramedullary lesions can also be no significant number of pupils large sample of patients encountered commission should pay particular attention to the history, signs of the base surface of the boat analysis, rather than emphasize the pupil is dilated boat , whether the shell can lead to diagnostic errors on.