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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical and postmortem findings of a case that had combined alcoholic pellagra encephalopathy and Wernicke disease are described. This 51-year-old malnourished and chronic alcoholic man presented with progressive mental deterioration, pellagra dermatitis, hypertonus of the neck and other musculatures, myoclonic jerks with bizarre involuntary movements, in addition to total external ophthalmoplegia and gait disturbance. After administration of multivitamins, including thiamine and nicotinamide, these neurologic abnormalities were dramatically improved in a few days. However, the patient died thereafter because of sepsis associated with pneumonia. Postmortem examination revealed marked abnormalities in CNS, characterized by diffuse atrophy of gray matter and widespread neuronal degeneration and characteristic central chromatolysis in pontine nuclei, dentate nuclei, cranial nerve nuclei in the brain stem, Betz cells of the cerebral cortex, and Clarke's column and anterior horn cells of the spinal cord. There were also atrophy and gliosis of the mammillary bodies, degeneration and vascular proliferation of periaqueductal gray matter, and massive gliosis around the third ventricle. These neuropathological changes were compatible with symptoms of both alcoholic pellagra encephalopathy and Wernicke's disease, but they were also strongly suspected on clinical grounds.
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PMID:Alcoholic pellagra encephalopathy combined with Wernicke disease. 188 55

A new inherited neuromuscular disease was identified in 4 patients (1 male, 3 females), offspring of consanguineous marriages, belonging to the same kindred. The proband was a 24-year-old female with history of ptosis and ophthalmoplegia since childhood and progressive intestinal pseudo-obstruction for the last 4 years of her life. A sural nerve biopsy showed axonal and demyelinating neuropathy. Muscle biopsies of pectoral and gastrocnemius revealed myopathic alterations with marked variation in muscle fiber size, atrophy of both fiber types and normal mitochondria. An upper gastrointestinal study showed barium in the stomach after 8 h and jejunal diverticula. Tests for absorption of fat, protein, carbohydrate, folic acid and vitamin B12 were normal. Serum levels of vitamin A and lipoproteins were also normal. The patient underwent partial gastrectomy and gastrojejunostomy. Postoperatively, she developed severe pancreatitis, sepsis, peritonitis and expired. Tissue samples from the proband and from her brother, revealed normal mucosa, but degeneration of smooth muscle of the stomach and small intestine. The myenteric plexus and vagus nerves were normal. The biochemical studies of contractile proteins (myosin, actin, tropomyosin) in the fresh and cultured smooth muscle cells of the proband obtained at the time of gastrectomy showed a 50-75% decrease in the synthesis of different contractile proteins. Turnover of contractile proteins and synthesis and turnover of collagen showed normal values. The reduction in synthesis of contractile proteins may account for the weak peristalsis and be a factor in the pathogenesis of the intestinal pseudo-obstruction.
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PMID:Inherited ophthalmoplegia with intestinal pseudo-obstruction. 668 98

Familial visceral neuropathy is a rare cause of chronic intestinal pseudo-obstruction. It is characterized by progressive destruction of the gastrointestinal myenteric plexus resulting in dysmotility and associated early satiety, post-prandial bloating, recurrent nausea and vomiting, abdominal distension, chronic diarrhea, weight loss, and malnutrition. In its varying forms, there may be neuronal destruction in other parts of the peripheral and central nervous system. We report on four siblings who presented in their third or fourth decades with initial clinical features of chronic intestinal pseudo-obstruction and eventual progressive diffuse neuronal disease, characterized by leukoencephalopathy and peripheral neuropathy. Within 5 yr of presentation, all four patients died from inanition and sepsis, despite aggressive nutritional support. Their clinical and pathological features are characteristic of familial visceral neuropathy of the autosomal recessive form. This presentation may represent a unique syndrome characterized by a tetrad of polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction.
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PMID:Familial visceral neuropathy as part of a diffuse neuronal syndrome: four fatal cases in one sibship. 817 58

The tegmentum of the midbrain is a complex area traversed by a number of anatomical and functional systems, including local circuits, ascending activating systems and descending fibers from the cerebral hemispheres. In the present paper we report on the case of a 67-year-old man who suffered a spontaneous central tegmental hemorrhage and was initially supposed to be in coma due to bilateral ptosis and lack of speech and initiative. By the second hospital week, however, he was shown to be able to respond to verbal commands, sit, stand and walk. He died in sepsis one month later. This case shows that the clinical diagnosis of coma may be misleading in certain patients in whom wakefulness is preserved, though concealed from a casual bedside examination due to abulia and ophthalmoplegia.
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PMID:[Spontaneous tegmental-mesencephalic hematoma: neurobehavioral aspects of the rostral third of the human brainstem]. 872 79

"Critically ill" polyneuropathy occurs in patients with artificial respiration. It is more frequent in men than in women. The most frequent underlying diseases, in the course of which CIP was found, were pneumonias, traumas and major surgery. In most cases sepsis and/or multiple organ dysfunction syndrome can be observed. Symmetrical motor polyneuropathy is the typical clinical pattern. In many cases the thoracic muscles are also involved. In some cases facial pareses and ophthalmoplegia may occur. Sensory disturbances are rather rare. The EMG often shows a myopathic pattern in addition to the neuropathic pattern. Morphologically, CIP is an axonal degeneration. The rate of lethality is high. In surviving patients there is a 50% chance of full remission.
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PMID:[Critically ill polyneuropathy]. 944 37

A 62-year-old man developed clumsiness, vertical ophthalmoplegia, right-side dominant parkinsonism, pyramidal signs, limb-kinetic apraxia and dementia. His brain MRI and SPECT revealed mild fronto-parietal atrophy and hypoperfusion predominately on the right side. At the age of 65, the patient died of sepsis. The duration of his illness was approximately 3 years. Clinical diagnosis was corticobasal degeneration (CBD). On neuropathological examination, there was no neuronal loss and many neurofibrillary tangles (NFTs) in the cerebral cortices. Basal ganglia and substantia nigra showed moderate to severe neuronal loss. And there were many NFTs and argyrophillic threads (threads) in the subthalamic nucleus and nuclei of the brainstem. These findings were almost compatible to those of PSP. However, the following findings were atypical for PSP, 1) mild atrophy and absence of gliosis in the tegmentum of the brain stem, 2) Mild neuronal loss in the subthalamic nucleus and the dentate nucleus without grumose degeneration. Furthermore, characteristic findings were noted on modified Gallyas-Braak stain. Numerous threads and many astrocytic plaques (AP) were shown in cerebral cortices, which were particular to CBD cases. So this case demonstrated neuropathological findings of both PSP and CBD. Therefore, it was difficult to classify this case as either PSP or CBD, and we diagnosed this case as a neuropathological overlapping case of both PSP and CBD.
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PMID:[A case manifested overlapping neuropathologic features of both progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD)]. 956 3

Isolated intracranial hypertension is a common manifestation of intracranial sino-venous thrombosis (ISVT). Markedly elevated intracranial tension presents with unusual features including cranial neuropathies and radiculopathy. We report two cases with ISVT, which presented with headache, papilledema, progressive visual loss, complete ophthalmoplegia and flaccid areflexic quadriparesis along with a normal sensorium. Magnetic resonance imaging (MRI) of the brain and cervical spinal cord showed no lesions that could account for the neurological deficits. Markedly elevated lumbar CSF pressure was noted in both cases. Nerve conduction study favored radiculopathy in one case and was normal in the other. Raised intracranial pressure was found to be the sole cause for the clinical manifestations. Visual impairment persisted in one patient despite lumbo-peritoneal shunting while the other died of septicemia. To our knowledge there are no previous reports of a syndrome comprising blindness, ophthalmoplegia and flaccid quadriplegia due to intracranial hypertension in ISVT.
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PMID:Blindness, ophthalmoplegia and extensive radiculopathy: an unusual clinical syndrome in intracranial sino-venous thrombosis. 1506 50

A 3 1/2-year-old boy presented with megaloblastic anemia and recurrent episodes of severe lactic acidosis and coma. At age 4 years, he developed sepsis and died; postmortem examination failed to show any gross abnormality in any tissue. Biochemical analysis of muscle showed decreased activities for all respiratory chain enzymes except complex II. Muscle histochemistry revealed diffuse cytochrome c oxidase deficiency. Southern blot analysis of mitochondrial DNA from muscle, liver, and blood showed a heteroplasmic single mitochindrial DNA deletion of 2.4 kb, which removed the genes for cytochrome c oxidase I and II and the transfer ribonucleic acid genes for serine and aspartic acid. Single large-scale deletions in mitochondrial DNA have been associated with Pearson's syndrome, Kearns-Sayre syndrome, and progressive external ophthalmoplegia. This patient's presentation is unusual and suggests an overlap between Pearson's syndrome and Kearns-Sayre syndrome.
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PMID:Mitochondrial DNA deletion in a child with megaloblastic anemia and recurrent encephalopathy. 1516 90

The purpose of the study was to enhance the efficiency of early diagnosis of orbital phlegmon, by examining its clinical picture and using instrumental studies. Sixty-three patients with orbital phlegmon were treated at hospital in 1985 to 2007. Its diagnosis employed ultrasound and X-ray studies. Orbital phlegmon was diagnosed in 30 patients with orbital injury and 33 patients with inflammatory diseases of the eyelids, face, nasal sinuses, and infection metastasis from septic foci. The disease was characterized by intoxication syndrome, eyelid inflammatory changes, chemosis, exophthalmos, and ophthalmoplegia. The following complications: neuritis, optic nerve ischemia, meningoencephalitis, brain abscess, cavernous sinus thrombosis, and sepsis were observed. Ultrasound and X-ray (computed tomography, magnetic resonance imaging) studies provide the diagnosis of the disease in early periods and timely medical and surgical treatments.
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PMID:[Orbital phlegmon: clinical picture, diagnosis]. 1920