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

At present, there are three major surgical approaches to Parkinson's disease (PD): (1) Ablative surgery (i.e. pallidotomy, thalamotomy); (2) deep brain stimulation (DBS) of the thalamus, internal globus pallidus (GPi) and subthalamic nucleus (STN); and (3) grafting fetal mesencephalic cells into the striatum. As a result of increasing understanding of the pathophysiology of the basal ganglia and the demonstration of surgical alleviation of experimental parkinsonism, surgery has regained a paramount importance in the management of PD. The aim of pallidotomy and DBS is to reduce the excessive inhibitory output from the GPi and substantia nigra reticulata (SNr). Pallidotomy and DBS of the STN or GPi aim to reverse the pathophysiological consequences of dopamine deficiency in PD, and should be considered entirely symptomatic treatments. The ideal candidates for pallidotomy are young patients in good general health in whom dyskinesias are the main reasons for disability. Patients with severe bilateral problems uncontrollable with present pharmacological tools are candidates for DBS. As yet, there are no formal data to help decide how to choose between GPi and STN stimulation. In our practice, patients are allocated to GPi stimulation when 'on' dyskinesias are extremely severe. In most other instances, we prefer to perform STN stimulation. At present there is almost no reason to decide for the thalamic stimulation since tremor is equally arrested by STN stimulation, which in addition improves all other features of PD. Equally the only indication for thalamotomy would be a patient with long-standing tremor as the main clinical manifestation, which can not be controlled with drugs. The proportion of patients in whom the thalamus will be the preferable target for either DBS or thalamotomy is small (less than 5%). Grafting aims to repair the nigrostriatal pathway and restore dopaminergic function in the striatum. In the future implants containing not only dopaminergic cells but also growth factors and a variety of other substances could become a method to not only functionally compensate the biochemical abnormalities of PD but also to arrest its progression. This technique is limited to a few centres around the world owing to the technical, logistical and ethical problems of obtaining and handling embryonic cells. At present, grafting of dopaminergic cells is perhaps best suited for patients with young-onset PD (less than 45 years old) who are at high risk of developing complications within a short time of beginning pharmacological treatment and in whom the idea of making lesions or implanting electrodes into the brain for decades seems less appealing. Consideration of surgery in any given patient should be weighed against the risks (about 1% mortality and 2-6% of severe morbidity-hemiplegia, cognitive deficit, speech problems, etc.) associated with these techniques. The development of better imaging methods and the growing expertise of multidisciplinary teams will undoubtedly make surgery for PD safer and more effective in the future.
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PMID:Surgical treatment of Parkinson's disease. 942 72

Moyamoya is a rare disease characterized by fibrous dysplasia of the internal carotid and proximal cerebral arteries, which has been described mainly in young Japanese. We present a case of Moyamoya disease with renal artery involvement in a young male patient with an African origin. A 15-year-old boy was referred to our hospital due to uncontrolled blood pressure, headache, somnolence, cognitive deficit and multiple lacunar infarcts in the computed tomography. Cerebral arteriography showed the absence of the normal vascular anatomy at the level of the circle of Willis. The intracranial vessels presented severe stenosis or were occluded and replaced by an extensive network of ectasic collateral vessels. Abdominal ultrasound examination identified asymmetric kidneys, and renal arteriography showed a tight stenosis of the ostium and proximal segment of right main artery, which was only partially relieved by balloon angioplasty. A saphenous bypass from aorta to the right renal artery re-established the renal blood flow. Blood pressure dropped after surgery and was controlled with low doses of diuretic and beta-blocker. After arteriography he presented right hemiplegia, with partial recovering in the following months. In conclusion, we described the first case of Moyamoya disease with concomitant renovascular disease in a young adult of African origin, an association that may be more frequent than usually suspected in clinical practice.
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PMID:Moyamoya disease associated with renovascular disease in a young African-Brazilian patient. 1146 61

Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients. In IHHS, children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. Patients with FIRES develop refractory epilepsy with severe cognitive deficit affecting the temporal and frontal lobe functions. The role of inflammation is hypothesized with a vicious circle involving inflammation and seizure activity facilitated by brain maturation putting them under the concept of "acute encephalopathy with inflammation-mediated status epilepticus."
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PMID:FIRES and IHHE: Delineation of the syndromes. 2400 Oct 74

The mechanisms underlying the Hemiconvulsion-Hemiplegia-Epilepsy syndrome remains unclear. The current proposed pathogenic mechanism is a neuronal injury induced by venous thrombosis and/or hypoxia. Children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. We report a 33 months old female child a case of hemiconvulsion-hemiplegia-epilepsy syndrome with right hemisphere unilateral brain edema and left sided hemiplegia and aphasia who presented with left upper extremities complex partial seizures with generalization to tonic clonic seizures and developed status epilepticus that posed diagnostic and therapeutic challenges. Progressive atrophy of the right cerebral hemisphere was noted after 3 months of follow up. Hemiconvulsion-Hemiplegia-Epilepsy syndrome should be suspected in a child with unilateral cerebral hemisphere brain edema and hemiplegia with cognitive deficit following status epilepticus to provide patients and families with an accurate prognosis regarding the subsequent development of epilepsy.
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PMID:Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in a Girl Presented with Complex Partial Seizures. 3035 4