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We present a rare case of thalamic germinoma with crossed aphasia in a dextral. A patient, 17-year-old righat-handed male, was admitted to Nippon Medical School Hospital with chief complaints of headache, abnormality of visual field and speech disturbance. There were pigmentations on the back of hand, foot and the perineum. Neurological examination revealed left homonymous hemianopsia, right slight degree of ptosis, left facial palsy, a mild paresis of the left upper extremity and motor aphasia. Right carotid angiography showed marked unrolling and midline shift of right anterior cerebral artery. CT scan revealed ring-like high density area in the right thalamic region, which was enhanced after constant infusion. Brain scintigraphy also showed an abnormal accumulation at the same site. The hen-egg sized tumor of 40 g. weight was almost totally removed by the right fronto-parietal craniotomy. The tumor was characterized histologically by the so-called two cell pattern with teratomatous components. As postoperative treatment local injection of adriamycine, irradiation and immunotherapy with picibanil were performed, and then left hemiparesis was markedly improved without sign of recurrence. Language evaluation was performed after operation. There were dysarthria, remarkable word amnesia, paraphasia and perseveration. Repetition was also impaired. His speech function was concluded to be a mixed type aphasia mainly composed of Broca's aphasia. The speech function of thalamus and crossed aphasia with dextrales were discussed.
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PMID:[A case of thalamic germinoma with crossed aphasia in a dextral (author's transl)]. 743 99

Mitochondrial myopathy, encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is one of the mitochondrial encephalomyopathies that has distinct clinical features including stroke-like episodes with migraine-like headache, nausea, vomiting, encephalopathy and lactic acidosis. We report a 27-year-old woman who presented with partial seizure, stroke-like episodes including hemiparesis, hemianopia and hemihypethesia, sensorineural hearing loss, migraine-like headache, and lactic acidosis. Brain computed tomographic scan showed encephalomalacia in the right parieto-occipital area and recent hypodensity in the left temporoparieto-occipital area with cortical atrophy. Muscle biopsy revealed ragged-red fibers and paracrystaline inclusions in the mitochondria. Genetic study revealed an A to G point mutation at nucleotide position (np) 3243 of mitochondrial DNA. External ophthalmoplegia and ptosis were also found during two exaggerated episodes in this patient. Therefore, the overlapping syndrome of chronic progressive external ophthalmoplegia in the MELAS syndrome is considered in this case. Furthermore, we also found carnitine deficiency in this patient and she was responsive well to steroid therapy. Muscle biopsy also revealed excessive lipid droplets deposits. Therefore, the carnitine deficiency may occur in MELAS syndrome with the A to G point mutation at np 3243. We recommend the steroid or carnitine supplement therapy be applied to the MELAS syndrome with carnitine deficiency.
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PMID:CPEO and carnitine deficiency overlapping in MELAS syndrome. 748 81

A prospective open study of botulinum toxin A treatment for patients with various movement disorders at Siriraj Hospital, Mahidol University was analysed to evaluate its efficacy. The grand total of 900 patients comprised of a) 592 patients (65.78 per cent) with hemifacial spasm; b) 92 patients (10.22 per cent) with occupational cramp; c) 79 patients (8.78 per cent) with blepharospasm and Meige syndrome; d) 72 patients (8.00 per cent) with spasmodic torticollis; e) 19 patients (2.11 per cent) with hemidystonia and generalised dystonia; f) 11 patients (1.22 per cent) with spasmodic dysphonia; g) 10 patients (1.11 per cent) with spastic hemiparesis; and h) 25 patients (2.78 per cent) with miscellaneous group (i.e. tics, Gilles de la Tourette, facial myokimia, benign fasciculation, etc.). The results of treatment for hemifacial spasm were classified as excellent in 486 patients (82.09 per cent), moderate improvement in 60 patients (10.14 per cent), mild improvement in 39 patients (6.59 per cent) and no improvement or worse in 7 patients (1.18 per cent). There were complications of mild transient facial weakness in 50 patients (8.45 per cent) and mild ptosis in 12 patients (2.02 per cent). The effect of botulinum toxin treatment lasted 3-6 months. In occupational cramp and spasmodic torticollis the good response rate was around two-thirds of all patients, whereas, blephalospasm, spasmodic dysphonia, spastic hemiparesis and tics responsed in 79-88 per cent of the patients. Botulinum toxin A injection is thus a simple, safe, and effective out-patient treatment for patients with various kinds of movement disorders but it is a costly therapy.
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PMID:Treatment of various movement disorders with botulinum A toxin injection: an experience of 900 patients. 756 52

From the literature and our own experience, 11 cases of hemorrhage or infarction of a pituitary adenoma associated with cardiac surgery have been identified over a 13-year period. Males outnumbered females by 10 to 1. Symptoms observed were headache, lethargy, confusion, obtundation, unilateral ptosis, meiosis, and opthalmoplegia involving cranial nerves III, IV, and VI, visual field deficits, and hemiparesis. Diagnosis in most recent cases has been confirmed with computerized tomography or magnetic resonance imaging. All patients received adrenocortical steroid therapy initially. Eight patients underwent transsphenoidal hypophysectomy and all survived. One patient underwent decompression craniotomy and died. Intracranial surgery was deferred in 1 patient who survived and in another who died of a massive stroke. Residual neurological deficits were noted to be either absent, minimal, or resolving in 7 of the 9 patients who survived their initial hospitalization. While numerous mechanisms have been proposed to explain the hemorrhage and necrosis of a pituitary adenoma during heart surgery, no direct cause has been clearly identified. Surgical treatment is commonly necessary since untreated pituitary apoplexy is often fatal. Transsphenoidal hypophysectomy with decompression is the preferred method of treatment with a low perioperative mortality and fairly good long-term prognosis.
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PMID:Pituitary adenomas complicating cardiac surgery: summary and review of 11 cases. 777 76

A nuclear oculomotor nerve syndrome is rarely caused by mesencephalic lesions. We describe 5 patients, 4 with unilateral syndrome (3 due to ischemia and 1 to mesencephalic hemorrhage) and 1 with bilateral nuclear ischemia of the third cranial nerve. These patients represent 0.2% of those admitted with cerebral vascular pathology over the last 12 years. Symptoms are consistent with descriptions of the anatomic organization of this nucleus, with peripheral paralysis of the contralateral superior rectus and possible bilateral involvement of the eyelid elevator and the pupillary constrictor muscles. A noteworthy symptom seen in these cases is supranuclear paralysis of the upward gaze on the side of the lesion, with ocular elevation achieved in response to oculocephalic stimuli in 2 cases and with Bell's synkinesis in 4. We analyze the nature of 22 published cases of unilateral nuclear damage and of 14 published cases of bilateral nuclear damage. Unilateral damage may or may not lead to ipsilateral pupillary involvement, uni- or bilateral eyelid ptosis, contralateral ocular hypotropia, and possible horizontal paresis of the contralateral gaze. Associated deficits are hemiparesis or crossed hemiataxia. Unresponsive pupils and bilateral ptosis associated with tetraparesis, bilateral ataxia and altered states of consciousness were seen with bilateral nuclear lesions. Infarction is the main cause (in 32 out of 41) and recovery of full ocular movement is uncommon.
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PMID:[Nuclear oculomotor nerve syndrome due to mesencephalic infarction or hemorrhage. Five cases and a review of literature]. 808 84

We describe a 15-year-old boy with full-blown mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and chronic progressive external ophthalmoplegia (CPEO). He presented with visual disturbance, hearing impairment, continuous partial epilepsy on the right aspect of the face, and right hemiparesis since the age of 13. Four months later, he experienced another strokelike episode with continuous partial epilepsy on the left hand. Serial computed tomographic scans revealed bilateral parieto-occipital hypodense lesions with gyral enhancement and an additional low-density lesion in the right frontal area 4 months later, respectively. Results of laboratory examinations disclosed lactic acidosis and mitochondrial myopathy with many ragged-red fibers. To identify the defective gene in mitochondrial DNA, a simple molecular test was performed by using restriction endonuclease Apa I. A transition from A to G was found at nucleotide position 3243 of the tRNA(Leu) gene. Interestingly, the patient also had marked external ophthalmoplegia and ptosis commonly found in patients with CPEO. Therefore, we suggest that ophthalmoplegia also occurs in the MELAS syndrome.
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PMID:Ophthalmologic manifestations in MELAS syndrome. 836 52

Bilateral ophthalmoplegia may be an unusual sign of vertebrobasilar ischemia. We report the cases of two patients (75 and 73 years old), who suddenly developed drowsiness, bilateral ophthalmoplegia with bilateral ptosis and mild right hemiparesis. In both patients, MRI revealed bilateral thalamic and midbrain infarcts, ECG showed the presence of atrial fibrillation and Doppler study of the extracranial and intracranial vertebral arteries found no significant alterations. Ischemia involving the midbrain and thalamic paramedian regions may cause bilateral ophthalmoplegia and consciousness disturbances. In these two cases, the most plausible etiologic mechanism was cardiac embolism, and the prognosis for bilateral ocular palsy was poor.
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PMID:Bilateral ophthalmoplegia: an unusual sign of the top of the basilar artery syndrome. 891 63

Medial medullary infarctions (MMI) were reported in less than 40 patients with satisfactory clinicotopographic documentation. We studied seven patients with MRI-proven acute MMI seen in two neurologic departments over a 5-year period (1990-1994). MMI represented less than 1% of ischemic strokes in the posterior circulation. Five patients had an infarction above the pyramidal decussation. All patients had contralateral hemiparesis and lemniscal sensory loss, accompanied by ipsilateral lingual palsy (Dejerine's syndrome) in three. Two patients had infarction below the pyramidal decussation, with ipsilateral hemiparesis and lemniscal sensory loss. Accompanying symptoms and signs of MMI were vertigo and nausea (n = 5), mild ipsi- or contralateral decrease in pain sensation (n = 6), headache (n = 4), ipsilateral limb ataxia (n = 6), contralateral truncal lateropulsion (n = 5), mild ipsilateral ptosis (n = 4), nystagmus (n = 4), dysarthria (n = 3), and somnolence (n = 2). Presumed causes of MMI were stenosis, occlusion or dissection of the ipsilateral vertebral artery (n = 5), and cardioembolism (n = 1). Outcome at 3 months was favorable in five patients. In conclusion, the clinical features of MMI are more heterogeneous than commonly thought, whereas its etiology seems fairly constant (vertebral artery disease).
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PMID:Medial medullary stroke: report of seven patients and review of the literature. 971 65

This case report describes a 15-month-old female who developed diffuse cerebral vasospasm after resection of a cerebellopontine angle primitive neuroectodermal tumor. The patient developed an acute dense left hemiparesis 16 days postoperatively with partial right ptosis. Initial magnetic resonance imaging and diffusion study were unremarkable, though a magnetic resonance angiography 1 day later demonstrated severe intracranial vasospasm of both carotid and vertebral arteries. The vasospasm was confirmed with cerebral angiography. The patient progressed to bihemispheric infarcts with laminar necrosis despite combination therapy with anticoagulation, pharmacological hypertension, hypervolemia, and nimodipine. The clinical course, radiographic, and pathological findings are presented.
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PMID:Diffuse cerebral vasospasm with ischemia after resection of a cerebellopontine angle primitive neuroectodermal tumor in a child. 997 76

Over a 3 yr period we performed colostomies in 13 paediatric perineal burn patients out of a total of 1544 patients admitted to our Burns Unit during that period. The mean total body surface area (TBSA) burn was 34% (14-65%); ten patients sustained fire burns and the remaining three hot water burns. We performed prophylactic colostomies in seven children, therapeutic colostomies (to counteract deep wound infection and septicaemia with gut-derived organisms) in five patients and one colostomy in a cerebral palsy child with a left hemiparesis. A sigmoid end-colostomy with Hartmann's closure of the distal segment was the preferred method of choice. In all children but one (died from multi-organ failure 13 days after admission) there was a marked improvement in the clinical appearance of the burn wounds and subsequent graft-take and healing. There was a change in the bacterial profile away from predominantly gut-derived Gram negative organisms to either Pseudomonas aeruginosa or no pathological organisms grown. Complications were few--two children suffered prolapse of their colostomy requiring manual reduction. We advocate diverting colostomies in a highly select group of paediatric burn patients in whom continual faecal soiling is threatening to both graft and life.
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PMID:The use of diverting colostomies in paediatric peri-anal burns. 1056 92


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