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A 28-year-old woman, with no past medical history, was admitted soon after a motor vehicle accident on March 1, 1990. On admission there were multiple small wounds in the right temporal region, but no wounds around the orbits. She was semicomatose with bilaterally fixed dilated pupils (6mm) and ptosis. The eyes were abducted bilaterally. No other cranial nerve palsy was noted. She moved four limbs spontaneously. No skull fracture was present on X-ray films. Cervical X-ray films revealed straightened cervical vertebral column but no fracture. Pelvic bone X-ray films demonstrated fracture and diastasis of the pubic bone. A computed tomography scan demonstrated a small subdural or subarachnoid hemorrhage in the right ambient cistern. A magnetic resonance imaging (MRI) carried out 12 days after trauma demonstrated bilateral small subdural hematomas under the cerebellar tentorium and contusional lesions of the bilateral medial temporal lobes. There were no abnormalities present in the brain stem. The patient was treated conservatively with diuretics and steroids. Disturbance of consciousness gradually improved until one month after the trauma, when she came close to being alert. Bilateral ptosis cleared after 3 months, and adduction of the eyes recovered after 6 months. Vertical eye movement improved a little in 6 months. Fixed 6mm pupils changed to fixed 4mm pupils in 6 months. Bilateral traumatic oculomotor palsy is a rarely described condition, and its mechanism remains conjectural. We discussed the mechanism of the injury and the site of the lesion.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Traumatic bilateral oculomotor nerve palsy: a case report]. 157 79

A 52-year-old woman with chronic progressive external ophthalmoplegia (CPEO) with familial hypercholesterolemia (FH) was reported. Her mother died from heart disease, and her elder sister has hypercholesterolemia with swelling of Achilles tendons. She had slowly progressive external ophthalmoplegia, bilateral ptosis, swelling of Achilles tendons since twenties. At 40 years of age, she was pointed out hypercholesterolemia. Physical examination was within normal limits except for bilateral swelling of Achilles tendons. Neurological findings showed bilateral ptosis, disturbance of eye movements, mild proximal muscle weakness and dysesthesia in bilateral hands. Routine laboratory findings were within normal limits except for high serum cholesterol level (512 mg/dl). In the biopsied muscle, there was mild variation in fiber size with several ragged-red fibers and focal cytochrome c oxidase defective fibers. Biochemical analysis of the biopsied muscle revealed normal values in the mitochondrial fraction. Southern blot analysis of the mitochondrial DNA (mtDNA) of the muscle disclosed mixed population of mtDNA, consisting of the normal one and partially deleted (4.9-kilobase). Southern blot analysis of the leukocytes from the patient against the cDNA of LDL receptor was normal at least using the restriction enzyme of BglII, XbaI, EcoRI, PvuII and BamHI. This case has CPEO with deleted mtDNA associated with familial hypercholesterolemia, which is caused to nuclear DNA abnormalities, and is thought to be an important case for us to study the relationship between deleted mtDNA and abnormal nuclear DNA in CPEO.
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PMID:[A case of chronic progressive external ophthalmoplegia associated with familial hypercholesterolemia]. 162 35

A young child with Hallervorden-Spatz syndrome is presented. She was well until 8 years of age when she lost interest in activities and her school performance declined. At age 11 years, she began having episodes of blepharospasm, accompanied by bilateral ptosis and occasional episodes of oculogyric crisis. By age 12 years, her motor coordination had declined and she began to exhibit evidence of dementia, dystonia, dysarthria, and tremor. Motor incoordination, dystonia, and tremor progressed until the patient was wheel-chair-bound. Multiple tests were performed, including metabolic studies, magnetic resonance imaging, bone marrow biopsy, and electron microscopy of the buffy coat. Both bone marrow and buffy coat revealed inclusions in the cytosomes which were granular and osmiophilic. To our knowledge, this is the third case report of inclusion bodies found in patients with manifestations of Hallervorden-Spatz syndrome. These findings suggest that obtaining a buffy coat and bone marrow biopsy may aid in the diagnosis of Hallervorden-Spatz syndrome and ultimately provide information regarding etiology.
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PMID:Osmiophilic deposits in cytosomes in Hallervorden-Spatz syndrome. 170 Jul 20

A 58-year-old woman with Garcin's syndrome caused by malignant meningioma is reported. She first noticed left facial hypesthesia followed by left visual disturbance with exophthalmos. CT scan showed an enhanced mass extending from the left cavernous sinus to the left orbit. Cranial x-ray revealed bone deformity around the orbit. The tumor was subtotally resected and its histological type was shown to be anaplastic meningioma. After the initial surgery the patient was left with hyposmia, mild ptosis and incomplete 6th cranial nerve palsy on the left side. After one and half years, not only palsy of those cranial nerves but also that of other unilateral cranial nerves became progressively apparent due to tumor regrowth. In total, 11 nerves (2nd-12th) were affected by the tumor, without long tract signs or symptoms of increased intracranial pressure. Radiation therapy carried out after the 2nd surgery was extremely effective and resulted in the recovery of lower cranial nerve function. The tumor was founded to have disappeared completely on the 2-year follow-up CT.
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PMID:[Radiosensitive malignant meningioma presenting Garcin's syndrome: a case report]. 173 27

An unusual presentation of acute megakaryocytic leukemia (AMKL) is reported in two young children. The first child had a 10-day history of ptosis of the right eyelid as the initial manifestation of AMKL, a clinical picture not previously described in this variant of leukemia. Computed tomographic scanning showed multiple intracranial mass lesions, and the diagnosis of AMKL was confirmed by immunophenotyping of bone marrow blasts. The second child had Down syndrome and received alkylating agents and radiation therapy for treatment of metastatic rhabdomyosarcoma of the orbit. She had AMKL as second malignancy. Both patients had acquired chromosome 21 anomalies in their leukemic blasts. The first patient, constitutionally normal, had an i(21q) in his leukemic blasts; the patient with constitutional trisomy 21 had tetrasomy 21 and additional chromosomal changes. The clinical symptoms and the results of morphologic, immunologic, and cytogenetic studies are discussed.
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PMID:Acute megakaryocytic leukemia in children. Clinical, immunologic, and cytogenetic findings in two patients. 183 41

A rare case of ischemic stroke related to Herpes zoster infection of the eye and documented arteritis in an HIV-positive patient is analyzed. The woman, aged 32, who was born in Angola and lived in Zaire, was diagnoses at the Hospital Universitario de Santa Maria, Lisbon. She presented with a 5-month history of sudden hemiplegia, 4 months after onset of herpes zoster ophthalmicus. Among extensive diagnosis tests, she was positive for HIV by ELISA and Western blot, hepatomegaly, and generalized lymphadenopathy. She has left Herpes zoster ophthalmicus with ptosis bulbi and mottled discoloration of the skin over the distribution of the 1st division of the left trigeminal nerve, and right spastic hemiparesis. Her helper T-cell count was 952/cubic mm, and her T-cell ratio was 0.9. She had anemia, hypoalbuminemia, positive serology for cytomegalovirus, Herpes simplex, Epstein Barr virus, and hepatitis B. She had no bacterial infections, but her stool contained Trichuris trichiura eggs and giardia lamblia cysts. Her cardiovascular system and cerebrovascular fluid were negative. Computed tomography of the head showed an old left capsular infarct. Cerebral angiography showed arteritis of the left choroidal artery with occlusion. She was treated with metronidazole and mebendazole, and had surgery for removal of the left eye with a prosthetic replacement. Strokes are common in AIDS patients, resulting from fungal infections, endocarditis, infectious or non-infectious emboli, or arteritis from herpes zoster infections. This is the 1st published case of hemiplegia and Herpes zoster in a European or African patient with HIV-1.
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PMID:Herpes zoster and controlateral hemiplegia in an African patient infected with HIV-1. 186 23

The entity of generalized livedo racemose and cerebrovascular bleeding disorders was introduced in 1965 by I. B. Sneddon describing 5 cases. it is not clear what role oral contraceptives and smoking play in the etiology of this syndrome. The case of a 44-year old multipara is described who had taken pills up to 1980 and smoked 5-10 cigarettes a day. In 1980 just before age 35 she suffered an apoplectic insult with hemisyndrome on the left side that she recovered from. An acute hypoglossal, and trigeminal paresis appeared on the left side. Computer tomogram showed a hypodense field in the area of both hemispheres of the brain. An audible mesosystolic click led to the diagnosis of suspecting cerebral embolism with mitral valve prolapse. Therapy was started with thrombocyte aggregation inhibitors. Although the prolapse could not be showed by echocardiography, the frontal mitral valve was slightly thickened. Another hospitalization in 1985 owing to a recurring attack of vertigo revealed higher blood pressure. She received betablocker treatment. In 1987 sudden weakness in the left arm and speech disorders ensued, and skin color alterations were manifest characteristic of generalized racemose livedo. Skin necrosis appeared on both toes. Sneddon syndrome was diagnosed, and full anticoagulation therapy was started with cumarin. The sensomotoric and speech symptoms receded only slightly. In 1988 a light cerebral insult occurred with the deterioration of the speech disorder. Laboratory finding showed immunoglobulin G (IgG) anticardiolipin antibodies (ACA) with 255 U/ml (normal range 0-10 U/ml), and normal IgM anticardiolipin antibodies with 8 U.ml (range of 0-10 U.ml). ACA has been detected in patients with lupus erythematosus and racemose livedo indicating the possible association of Sneddon syndrome with systemic lupus erythematosus.
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PMID:[Sneddon syndrome]. 187 23

A 42-year-old woman was admitted for complaining of gait disturbance lasting for 2 years. She had a past history of hearing loss and bilateral ocular ptosis since her age of 21 years. Neurological examinations revealed severe external ophthalmoplegia, ptosis and muscular weakness of neck and extremities, but no dementia. Laboratory examinations showed high level of serum CK and slight elevations of CSF protein, lactate and pyruvate. The QT interval on ECG was prolonged. The EEG finding was slightly abnormal. The neurogenic pattern on EMG was noted. Muscle biopsy was performed at the quadriceps muscle. Its specimens demonstrated mixed changes of muscular atrophy and ragged red fibers. From these findings, Kearns-Sayre-Shy syndrome was diagnosed. It was noted in this case that brain MRI (T2-weighted image) revealed bilateral diffuse high signal intensity areas on cerebral white matter and brainstem. It seems that the lesions of central nervous system may progress subclinically for many years in cases of KSS, as our case shows.
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PMID:[A case of Kearns-Sayre-Shy syndrome with abnormal signal intensity on MRI in cerebral white matter and brainstem]. 193 67

The disturbance of visual perception associated with nystagmus is a rare phenomenon. This is a case of a 61-year-old woman who developed progressive right hemisensory deficit, left facial sensory deficit, vertigo, staggering to the left, left ptosis, vertical diplopia, and ataxia of the left upper extremity. She had rotatory nystagmus in primary position, which increased in amplitude with left gaze. The above signs and symptoms were consistent with lateral medullary syndrome. During her rehabilitation, the patient complained of visual disturbances typical of oscillopsia. These disturbances, or illusions, are compensatory mechanisms for nystagmus and its resultant retinal error. The purpose of this case presentation was to study the pathophysiology underlying oscillopsia in patients with nystagmus and to stimulate awareness of such visual disturbances in stroke patients.
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PMID:Visual illusions in a patient with lateral medullary syndrome. 199 Oct 18

A 17-year-old woman with mitral and tricuspid valve prolapse and myxomatous degeneration presented puerperal infection by Staphylococcus aureus with clinical picture of sepsis and multiple septic embolism (right eye, left thumb, spleen, and left calf). She underwent total hysterectomy on the 10th day postdelivery and right eye enucleation on the 16th. Temporary total AV block occurred on the 14th day with temporary external pacing during the next couple of days. Acute endocarditis with acute mitral regurgitation was diagnosed on the 13th day, demanding immediate valve replacement. On the 46th day she developed moderate tricuspid valve regurgitation due to another episode of endocarditis. Final clinical discharge took place on the 62nd day after antibiotic therapy completion.
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PMID:[Staphylococcus aureus endocarditis in a puerperal woman with mitral and tricuspid valve prolapse]. 209 20


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