Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors experienced a case of histiocytosis X with a large intracranial mass resulting in a convulsive seizure. The patient showed left exophthalmos and a skin rash one year and two months after birth. Histiocytosis X was diagnosed from a skin biopsy, and predonine, endoxan and vincristine were administered. The rash disappeared, but the exophthalmos remained. At the age of two years and nine months, punched-out lesions appeared in the skull and 4,000 rads of radiation was applied. Thereafter, the exopthalmos persisted but there was no particular problem in the course. However, a convulsive seizure with fever suddenly appeared at nine years and ten months of age and the patient was hospitalized. At the time of admission, the general condition was good and there were no abnormalities in neurological tests. In neuroradiological examinations, a calcified and poorly vascularized mass 8 cm in maximum diameter was found to occupy the left middle cranial fossa. Chondrosarcoma was strongly suspected from these findings, but there was also symmetrical thickening of bone cortex in the peripheries of the long bones of the extremeties which appeared to be the recovery process from bone destruction caused by histiocytosis X. Therefore, the formation of an intracranial mass by histiocytosis X was diagnosed and surgery was performed. When left osteoplastic fronto-temporal craniotomy was performed, the mass was found to be raising the temporal lobe and it could be easily separated from the surrounding tissue. However, these was firm adherence to dura mater of the middle cranial fossa (especially that of the superior orbital fissure). Histologically, there were many cells with small nuclei, no polymorphism, abundant and clear cytoplasm which were darkly stained and slightly atypic. These findings matched those for histiocytosis X. Cases of histiocytosis X rarely show symptoms of the central nervous system or infiltration of the central nervous system. Only 31 such cases were seen in the literature investigated by the authors. Neurological symptoms include pyramidal symptoms such as hemiparesis and impairment of the cranial nerves, particularly paresis of the optic, trigeminal, facial and acoustic nerves. Convulsive seizures were seen in only five cases including the one reported here. It is also rare for intracranial masses to be formed in cases of histiocytosis X and only six cases, including the authors', have been found with masses of a maximum diameter of more than 5 cm.
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PMID:[Case of histiocytosis X with a large intracranial mass]. 660 36

Brain tumors are, after leukemias, the most frequent fatal neoplasms of infancy. The clinical features and symptoms are often markedly different from those observed in the adult forms, according to the peculiar anatomy and behaviour of the child. Persistent headache, vomiting, astenia , behavioral alterations may be the precocious findings. Later, some more specific and suggestive signs such as strabismus, dyplopia , fast head size increase, funduscopic alterations, ataxia, paresis and nystagmus may be observed. On their appearance a prompt diagnostic work-up should be performed. The tumors of the posterior fossa (cerebellar astrocytoma and medulloblastoma, brainstem glyoma , hependimoma in decreasing order of frequency) generally cause precocious symptoms because of the small dimension of the subtentorial space; the presence in this region of several fundamental nuclei and pathways may explain how also small tumors may cause severe deficits. Supratentorial tumors (astrocytoma, malignant glyoma , hependimoma , craniofaringioma ) often show a more prolonged latency and may begin with signs of endocranial hypertension, seizures, or sometimes with hormonal troubles according to the involved anatomic structures. Hypothalamic astrocytoma is responsible of an extreme weight loss as far as to a cachetic status, due to the hyperincretion of GH. Finally, plexus papilloma, dermoid, optic nerve glyoma , oligodendroglyoma , germinoma, teratoma are responsible of a small number of child brain tumors, with different localization and symptomatology.
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PMID:[Clinical course of brain tumors in childhood]. 673 95

Three principal diagnostic problems were encountered in a group of 81 elderly patients with epileptic seizures: Post-ictal paresis (Todd's palsy) occurred in 13 patients (16%), of whom seven had evidence of past or present cerebral infarction, and six no such evidence. The paresis lasted up to 4 days, and could be confused with recurrent infarction or a transient ischaemic episode. Ictal and post-ictal confusional states of 24 h or more duration (up to 7-8 days) were noted in 11 patients (14%). They occurred in association with normal intellectual function as well as with pre-existing dementia, and required to be distinguished from other causes of delirium. Two patients presented paroxysmal sensory phenomena of ictal type. The differential diagnosis of episodic pain in hemiplegic limbs is discussed.
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PMID:Epileptic seizures in the elderly: II. Diagnostic problems. 680 29

Only rarely will a child continue to have focal neurologic findings, such as paresis, for as long as 8 years after LAC encephalitis. Most of the other focal neurologic findings, such as Babinski reflexes, pathologic reflexes, aphasias, choreas, dysarthrias and ataxias, resolve completely. Abnormal electroencephalographic findings during the acute period were note in 86-100% of the cases. On 1-8 year follow-up, EEG abnormalities were noted in approximately 33% of the subjects tested. Seizures are present at high frequency during acute illness and recurrent seizures may occur in 6-13% of the cases 1-8 years after infection. The cognitive and intellectual functioning of children following LAC encephalitis is not significantly different from that of the normal population. As a group, LAC encephalitis victims also function in the normal range in terms of their academic performances. There are individuals, however, who will have suffered permanent destructive lesions resulting in lowered IQ and lowered school performance. Behavioral abnormalities are difficult to measure and when present, difficult to ascribe to a definitive cause.
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PMID:Clinical aspects of La Crosse encephalitis: neurological and psychological sequelae. 686 35

From 1972-1974, 228 children began treatment for acute lymphocytic leukemia and were prospectively assessed for neurologic complications. After CNS irradiation (2,400 rad) and intrathecal methotrexate (MTX), they received weekly intravenous maintenance therapy with MTX alone (40-60 mg/m2; 20 patients) or MTX (10-30 mg/m2) with other drugs (208 patients). Signs of leukoencephalopathy appeared in 11 children (nine without CNS leukemia) after 4-15 months of IV MTX alone, and included lethargy, seizures, spasticity, paresis, drooling, and dementia. Before or during the clinical onset, EEG frequencies slowed (all ten patients tested). Radionuclide scans showed periventricular accumulation of 99mTc (9/11 patients) and remained abnormal for greater than or equal to six months in eight patients. Cranial computed tomograms or neuropathology findings (five patients each) demonstrated leukoencephalopathy (nine patients) and radiation-related microangiopathy (ten patients). Severe neurologic and neuropsychologic dysfunctions were present in four long-term survivors.
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PMID:Progression of methotrexate-induced leukoencephalopathy in children with leukemia. 693 56

Two examples of intracerebral neurilemmoma occurred in the absence of von Recklinghausen's disease. The first case was unique because the patient was clinically asymptomatic, and the second case was unusual with a combination of oligodendroglioma and neurilemmoma in the same mass. The latter lesion arising in persons without stigmata of neurofibromatosis has not been described. The nature of nerve sheath neoplasms in both instances has been verified by electron-microscopy. Clinical features of this rare neoplasm differ from those of acoustic or other cranial nerve tumour, particularly in relation to age and sex. Intracerebral neurilemmoma has been found most often in the first two decades of life and more often in males. Seizures and paresis, although non-specific are common signs and symptoms. Probable histogenesis of this neoplasm from perivascular elements in brain has been suggested.
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PMID:Intracerebral neurilemmoma. 733 5

Occasionally patients in the stroke age-bracket over 40 have unexplained transient cerebral ischemic attacks in association with normal cerebral angiograms. From this group 120 have been collected in whom the transient episodes resembled the neurological accompaniments of migraine. According to symptoms, the patients were categorized as follows: Visual accompaniments (patients with only ordinary scintillating scotoma were excluded), 25; visual and paresthesias, 18; visual and speech disturbance, 7; visual, and brain stem symptoms, 14; visual, paresthesias, and speech disturbance, 7; visual, paresthesias, speech disturbance and paresis, 25; recurrence of old stroke deficit, 9; miscellaneous, 8. In establishing the diagnosis angiography is advisable in all but classical cases. Typical of migrainous accompaniments are the buildup and migration of visual scintillations, the march of paresthesiae, and progression from one accompaniment to another, characteristics that do not occur in thrombosis and embolism. Diagnosis facilitated when 2 or more similar episodes have occurred or migraine-like scintillations are present. Headache occurred in 50% of cases. Other cerebrovascular processes, coagulation disorders, and cerebral seizures must be ruled out.
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PMID:Late-life migraine accompaniments as a cause of unexplained transient ischemic attacks. 738 4

Cytokines are now recognized to play important roles in the physiology of the central nervous system (CNS) during health and disease. Tumor necrosis factor alpha (TNF-alpha) has been implicated in the pathogenesis of several human CNS disorders including multiple sclerosis, AIDS dementia, and cerebral malaria. We have generated transgenic mice that constitutively express a murine TNF-alpha transgene, under the control of its own promoter, specifically in their CNS and that spontaneously develop a chronic inflammatory demyelinating disease with 100% penetrance from around 3-8 weeks of age. High-level expression of the transgene was seen in neurons distributed throughout the brain. Disease is manifested by ataxia, seizures, and paresis and leads to early death. Histopathological analysis revealed infiltration of the meninges and CNS parenchyma by CD4+ and CD8+ T lymphocytes, widespread reactive astrocytosis and microgliosis, and focal demyelination. The direct action of TNF-alpha in the pathogenesis of this disease was confirmed by peripheral administration of a neutralizing anti-murine TNF-alpha antibody. This treatment completely prevented the development of neurological symptoms, T-cell infiltration into the CNS parenchyma, astrocytosis, and demyelination, and greatly reduced the severity of reactive microgliosis. These results demonstrate that overexpression of TNF-alpha in the CNS can cause abnormalities in nervous system structure and function. The disease induced in TNF-alpha transgenic mice shows clinical and histopathological features characteristic of inflammatory demyelinating CNS disorders in humans, and these mice represent a relevant in vivo model for their further study.
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PMID:Spontaneous inflammatory demyelinating disease in transgenic mice showing central nervous system-specific expression of tumor necrosis factor alpha. 747 82

Certain behaviors that occur during a complex partial seizure (CPS) are useful in lateralizing the side of seizure onset. In 5 (5.3%) of 94 consecutive patients with partial epilepsy, we observed ictal unilateral arm and hand paresis during 27 of 34 CPS. In all these seizures, this behavior occurred contralateral to an epileptogenic temporal lobe, as determined by video-EEG monitoring and surgical outcome. In 5 of the 27 seizures, an observer demonstrated that the paretic arm and hand were flaccid. None of these patients had postictal (Todd's) paralysis. In most of the seizures, the arm ipsilateral to seizure onset had simultaneous purposeful movements or automatisms, sometimes with awkward posturing. Ictal unilateral paresis is distinctly different from ictal dystonia or postictal paralysis and consistently lateralizes seizure onset to the contralateral temporal lobe. Recognition of this particular ictal behavior and comparison to other simultaneous behaviors can aid in the lateralization and possibly localization of the epileptogenic zone.
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PMID:Ictal contralateral paresis in complex partial seizures. 755 83

The Sneddon's syndrome consists of neurologic manifestations associated to the presence of livedo reticularis and cyanosis of the extremities. The pathological process is an endothelial obliteration of arterioles, leading to a reticular appearance of the skin, despite the environment temperature. The authors present three new cases, caucasian males with 7, 16 and 54 years of age. The youngest started with hemilateralized motor seizures and showed a porencefalic area in the CT scan. The oldest had livedo reticularis, acrocyanosis and started with hemilateralized motor seizures, and a hemiparesis as sequela; CT scan with parasagittal infarct and occlusion presented of one anterior cerebral artery on angiography. The third patient started with hemifacial seizures, developed a labioglossolaringeal paresis and dysarthria as sequela; CT scan and MRI showed multiple infarcts, with multiple occlusions of cortical branches on angiography. The skin biopsies showed endothelial vascular hyperplasia in all cases. Only one (54 years old) patient had a positive IgG antiphospholipid antibodies. The Sneddon's syndrome seems not to be so rare and have to be considered in the etiological investigation of cerebral infarcts, mainly in young people.
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PMID:[Sneddon syndrome. Report of 3 cases]. 757 13


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