Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Study of a 4 1/2-year-old boy with the unusual combination of acute infantile hemiplegia, ectopia lentis and the absence of homocystinuria showed large amounts of abnormal sulfur-containing metabolites (sulfite, thiosulfate and S-sulfocysteine) in the urine. Sulfite and S-sulfocysteine were also present in the plasma. His inorganic sulfate excretion was only 50 per cent of total sulfur, as compared with 75 to 95 per cent by controls. Loading with L-cysteine hydrochloride and L-methionine further increased the excretion of sulfite and thiosulfate, but not inorganic sulfate excretion. Sulfite oxidase activity in skin fibroblasts average 1.07 nmol of cytochrome d reduced per milligram of protein per minute in control lines; it was not detectable (less than 5 per cent) in the patient. Activity was reduced in both parents (0.50 in the father and 0.32 in the mother)--compatible with autosomal recessive inheritance. Good biochemical responses to a low sulfur amino acid diet suggest that early treatment may benefit the patient.
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PMID:Sulfite oxidase deficiency. Biochemical and clinical investigations of a hereditary metabolic disorder in sulfur metabolism. 30 14

This paper presented a case of a right-handed male who showed a right hemiplegia without aphasia and apraxia. He lost the ability to write with the left hand. A 56-year-old right-handed man, who had a daughter of left-handedness, was sent to our hospital with a homonymous hemianopsia, facial weakness, spastic hemiparesis and sensory disturbance in the right side. CT scan revealed an infarction in the territory of the left middle cerebral artery. On a month after the onset, he was alert and oriented. His speech was normal and verbal comprehension was intact. Although he neglected the right side of the page, he could read and comprehend it correctly. In contrast with his normal abilities to speak, comprehend, and read, difficulties in writing were prominent. Spontaneous writing with the left hand was extremely poor, and he even had difficulty writing his own name. His dictation was also poor, but his writing improved with copying letters. Agraphia had seen even after USN was recovered. Analysis of this case suggested the presence of the dominance for speech, comprehension, and praxis in the intact right hemisphere, and writing center in the damaged left hemisphere.
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PMID:["Left unilateral agraphia with right hemiparesis" after occlusion of the left middle cerebral artery]. 141 44

A four-year-old child was admitted to hospital with an infarct of the right middle cerebral artery involving the frontoparietal area. His symptoms included left hemiplegia and aphasia. After two weeks, he had hemiparesis, word-finding and naming problems and enuresis. A year later he demonstrated elective mutism at school, had attention and short-term memory impairments, occasional enuresis and an average IQ. He was shy and withdrawn; this is interesting, since depression is usually associated with left-hemispheric lesions. It is suggested that an early period of mutism should be included among the criteria for the study of crossed aphasia in children, as this is a common occurrence in such cases. Even after recovery of speech, impairments in attention and academic skills may persist.
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PMID:Crossed aphasia in early childhood. 169 99

We report a 9 year-old boy with MELAS. High dosed oral thiamine administration and high fat diet induced remarkable neurological and biochemical improvement. His mother had episodic headaches and hemiplegia, probably MELAS. He complained muscle weakness and repeated episodes of vomiting started from 2 years of age. High levels of serum lactate and pyruvate were recognized, but with no metabolic acidosis. He developed generalized muscle weakness, growth retardation, generalized convulsions and stroke-like episodes at 5 years old. Optic nerve atrophy and mental retardation gradually appeared. A muscle biopsy at 5 years old revealed numerous ragged-red fibers with excess accumulation of lipid droplets and glycogen particles. Scattered fibers had no cytochrome c oxidase (CCO) activity representing focal CCO deficiency. An electron microscopy showed markedly increased number of giant mitochondria filled with markedly proliferated complicated cristae. Pyruvate dehydrogenase complex level in the fibroblasts was within normal ranges. Serum carnitine level was normal. With oral administration of thiamine hydrochloride (1000 mg) and high fat diet (60-70%), muscle weakness improved, and lactate and pyruvate levels in the serum reduced to normal ranges, whereas the mental deterioration, muscle atrophy, pes cavus progressed very slowly. He died from cardiac and renal failures at 9 years old. Autopsied muscles showed a marked decrease in cytochrome c oxidase activity (biochemically 12.8% of the normal level), and almost all muscle fibers had no cytochrome c oxidase activity histochemically. The progression of the MELAS was probably in parallel with the decrease in CCO activity.
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PMID:[A case of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) with progressive cytochrome c oxidase deficiency]. 255 13

We reported a 72-year-old male with ischemic oculopathy due to ophthalmic artery stenosis followed by ipsilateral border zone infarction due to internal carotid artery stenosis. The patient had history of hypertension and diabetes mellitus. He had severe headache and visual disturbance of the right eye. He was diagnosed right neovascular glaucoma and left diabetic retinopathy (simple type), and received diuretics, beta-blockade and other anti-hypertensive drugs. One month later, he noticed left mild hemiparesis in a morning, and he experienced progression of left hemiparesis over a week. He was admitted to our hospital on the 11th day. He showed left complete hemiplegia, left sensory disturbance, anosognosia and left unilateral spatial neglect. His right eye was diagnosed neovascular glaucoma but left eye was normal. The 5th days CT showed low density area in the right terminal zone and bilateral periventricular lucency. At the same area, the 46th days MRI showed high intensity area in the T2-weighted image and low intensity area in the T1-weighted image. Cerebral angiography performed on the 33rd day, disclosed severe kinking at the cervical segment and 50% stenosis at the intracavernous segment in the right internal carotid artery, and 90% stenosis and post-stenotic dilatation of the right ophthalmic artery. Left internal carotid artery had each 60% stenosis at the cervical segment and the intracavernous segment. Left ophthalmic artery had severe stenosis from its beginning to distal part. This infarction was considered berder zone infarction by it's localization (terminal zone) and internal carotid artery stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of ischemic oculopathy followed by border zone infarction]. 258 88

We reported a 41-year-old male with paramedian thalamic and midbrain infarcts due to cerebral embolism from bilateral pulmonary arterio-venous fistula and primary medullary hemorrhage. The patient had an episode of sudden onset consciousness disturbance with left Weber's syndrome (right hemiplegia and left oculomotor palsy) and vertical gaze palsy at age of 23. He noticed numbness in the left hand and the left half body under clavicular when he had got up in a morning at age 41. He had headache and left tinnitus on second and third days, and on the 3rd and 4th days, he experienced nausea. He had severe hiccup persisting from the 6th to the 13th days. The 23rd days he was admitted to our hospital. He showed dysesthesia and paresthesia in left half body under clavicular, dysesthesia in left hand and vertical gaze palsy and convergence disturbance. MRI performed on the 18th and 24th days, disclosed hyperdense mass in T1 and T2-weighted images in dorsal site of medulla, but the 70th days MRI showed no abnormal lesions. Therefore we diagnosed the high intensity mass as primary medullary hemorrhage. Cerebral angiography showed no abnormal vasculature. Many members of his family had history of sever nasal bleeding. He had skin hemangioma and mucosal hemangioma in esophagus, stomach, colon and rectum, and bilateral pulmonary arterio-venous fistula which had been operated at age 39. His mother also had skin hemangioma and pulmonary arterio-venous fistula. Therefore this family was diagnosed Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia). MRI also disclosed multiple cerebral infarctions in bilateral thalamus, left cerebral peduncle and left cerebellar hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Rendu-Osler-Weber syndrome presented paramedian thalamic and midbrain infarcts and primary medullary hemorrhage: a case report]. 269 33

Alternating hemiplegia in childhood (Verret, 1971) is a disorder presenting with frequent episodes of alternating hemiplegia from early infancy. We report a patient with this disorder, along with a pathophysiological study and a discussion about the available therapies for this disorder. The patient, an 11-year-old boy, visited our hospital with episodes of alternating hemiplegia from early infancy. His family history showed that many members suffered from migraine. He was born with asphyxia. Mental and motor developmental delays were seen from early infancy. The hemiplegic episodes with ipsilateral facial palsy had occurred frequently from early infancy. The episodes were frequently induced by emotional stress. The duration of hemiplegia varied from 10 minutes to 3 days. From the age of 11 years, he had begun to have migrainous attacks with or without hemiplegic episodes. Neurological examination revealed slight muscle hypotonia, choreoathetosis and dystonic movements induced by locomotion, positive Myerson sign, increased deep tendon reflexes and Babinski reflex. CAG, VAG and CAT revealed normal findings. EEG revealed diffuse generalized slowing during hemiplegic episodes. Measurement of regional cerebral blood flow (CBF) by 133Xe inhalation method revealed a slight decrease of bilateral CBF during a quadriplegic episode. Positron emission tomography using C15O2 revealed a slight decrease of CBF at the insula, putamen and claustrum of the left side during a right sided episode. Increased excretion of urinary 5-HIAA was seen during one episode. From our clinical and laboratory findings, we think this disorder may be a special type of migraine. Therapeutic trials of diazepam and flunarizine were both effective, but the initial effectiveness was decreased after 5 months.
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PMID:[A patient with alternating hemiplegia in childhood]. 273 28

Fatal traumatic thrombosis of the left internal carotid artery occurred in a 38-year-old man following minor blunt cervical trauma during an altercation. There was no external injury observed on the head, face, or neck. Neurologic deficit developed soon after the injury, which progressed to right hemiplegia. His condition deteriorated and he expired on the sixth hospital day. The gross and microscopic findings of the internal carotid artery are presented and the significance of minor cervical blunt trauma and the possible mechanism for the vascular lesion are discussed.
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PMID:Fatal thrombosis of internal carotid artery following minor blunt trauma to the neck. 239 72

Dilated cardiomyopathy (DCM) is defined as a syndrome of dilated ventricles with gross impairment of ventricular systolic function. However, few reports on perioperative management of DCM were obtainable. This paper describes perioperative management of two patients with DCM. A 72-year-old man, whose DCM had been treated with medication, was planned for gastrectomy. His cardiac signs indicated NYHA class II. Cardiac function, evaluated prior to the surgery with echocardiography to determine an operative indication, turned out to be well compensated. Minimal cardiac derangements were anticipated perioperatively. The procedure was carried out under general anesthesia with neuroleptanalgesia utilizing butorphanol and vasoactive agents. No circulatory complications were observed throughout the surgery. A 66-year-old man, who had long-standing heart disease, hemiplegia and hydronephrosis due to ureteral stone, was planned for percutaneous nephrolithotomy. However, the planned surgery was withdrawn because he had DCM with minimal compensatory function and perioperative cardiac derangements were anticipated according to the categorized data classified with echocardiography. We conclude that preoperative assessment of cardiac function is essential to minimize perioperative cardiac derangements in patients with DCM.
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PMID:[Perioperative management of patients with dilated cardiomyopathy]. 273 70

A 74-year-old right-handed man with multiple cerebral infarction who presented with dementia simulating dementia of Alzheimer type (DAT) is reported. He had been well until April 20, 1987 when he developed transient right hand palsy lasting overnight. Eleven days later, he became confused, disorientated, and amnestic. He was admitted to this hospital on June 8. Physical examination revealed hypertension (170/90mmHg). On neurological examination, his consciousness was clear but he was demented. He showed disorientation, amnesia, and urinary incontinence. His most prominent symptom was disturbance of speech, including fluent aphasia and alexia with agraphia. Additionally, he showed ideomotor apraxia, construction apraxia, right-left agnosia, finger agnosia, and acalculia. On July 9, he had a transient attack of right hemiplegia with confusion. The brain CT scan performed on admission was unremarkable except for cavum septi pellucidum and a small low density area in the right basal ganglia. However, single photon emission computed tomography (SPECT) by 123I-labeled N-isopropyl-p-iodoamphetamine disclosed hypoperfusion of the cerebral blood flow in the border zones of the temporoparietal and frontal lobes on the left. A follow-up brain CT scan taken one month later demonstrated low density in the new areas corresponding to hypoperfusion shown by SPECT. Although the clinical features of the present case resembled those of DAT, dementia in this case was regarded as the result of multiple cerebral infarction since it occurred acutely with mild motor deficits, and brain CT scans and SPECT showed lesions indicating focal cerebral ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Multi-infarct dementia clinically simulating dementia of Alzheimer type. A comparison with angular gyrus syndrome]. 278 20


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