Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 48-year-old man, who took by mistake a sip of ointment containing dichloroethane, survived, and showed a course of two phases of toxic symptoms. After an initial narcosis and an interval with few pathological symptoms seizures, myoclonia and somnolence occurred. Irreversible final disturbances were lasting mental defects, cerebellar dysarthria, ataxia, and hydrocephalus. Concomitant diseases were acute liver dystrophy, nephropathy, and anemia. The clinical picture of dichoroethane posoning is outlines, the pathogenesis of this particular cerebral lesion described, and the therapy discussed.
 ...
PMID:[Dichloroethane poisoning with myoclonic syndrome, seizures and irreversible cerebral defects (author's transl)]. 122 Jun 46

Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation or at necropsy caused by cutting outwards of the CSF under pressure from the fourth ventricle into the medulla. These should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a previously established syringomyelia. Clinical analysis of 54 patients suggests that bulbar features are most often found with neither of the above mechanisms but are due to the effects of pressure differences acting downward upon the hind-brain with consequent distortion of the cerebellum and brainstem, traction on cranial nerves or indentation of the brain-stem by vascular loops. The commonest symptoms in the 54 patients were headache (35), vertigo (27), dysphonia or dysarthria (21), trigeminal paraesthesiae (27), dysphagia (24), diplopia (16), tinnitus (11), palatal palsy (11) and hypoglossal involvement (11). Careful attention to hydrocephalus is advisable before craniovertebral surgery, but the decompression of the hindbrain and the correction of craniospinal pressure dissociation remains the mainstay of surgical treatment. The results of careful surgery are good, 45 of the 54 cases reported improvement. Most of the reported deterioration occurred in a few patients who did conspicuously badly.
 ...
PMID:Syringobulbia: a surgical appraisal. 147 91

We describe 2 normotensive sisters presenting slowly progressive dementia associated with acute or subacute focal neurological symptoms, unilateral or bilateral motor signs, and dysarthria. Their father, who died in the seventh decade, had a similar clinical picture. Computerized axial tomography (CT) scan of the head showed symmetrical hypodensities in the periventricular white matter and mild to moderate hydrocephalus. In these patients a diagnosis of Binswanger's disease was based on the clinical features supported by white matter changes on CT scan. Our study suggests that genetic factors may play a role in the etiology of Binswanger's disease.
 ...
PMID:Slowly progressive familial dementia with recurrent strokes and white matter hypodensities on CT scan. 159 74

A rare case of cavernous angioma located in the fourth ventricular floor occurred in a 44-year-old female complaining of occipital headache, vomiting, diplopia, and dysarthria. Computed tomographic scans demonstrated a high-density area in the fourth ventricle and slight hydrocephalus. Magnetic resonance (MR) imaging showed a mixed intensity mass on T2-weighted images and high- or isointensity regions on T1-weighted images. The tumor was totally removed and histologically diagnosed as cavernous angioma. Postoperatively, ataxic gait, nausea, and vomiting disappeared gradually. MR imaging was useful to accurately evaluate the anatomic relationship between the lesion and the brainstem.
 ...
PMID:Cavernous angioma in the fourth ventricular floor--case report. 171 36

Distinct chronic posttraumatic syndromes, ascribed to neurological deficits of patients suffering severe head injuries and being in prolonged coma, are much less frequently encountered in the literature than acute traumatic syndromes. The major components of the posttraumatic midbrain syndrome, resulting from compressive necrosis or vascular infarction at the midbrain level, are ipsilateral cerebellar signs (the predominant one being intention tremor), contralateral pyramidal signs (the predominant one being a spastic-dystonic hemiparesis), dysarthria, and mild to moderate intellectual impairment. Significant bilateral cerebellar dysfunction following head injury, without pyramidal, extrapyramidal, or pseudobulbar signs, constitutes a posttraumatic cerebellar syndrome. Its most disabling component, namely posttraumatic intention tremor, may be alleviated by thalamotomy. Following severe closed head injury, an infrequently encountered posttraumatic entity of dystonic hemiplegia or hemiparesis, which may be alleviated by thalamotomy, can occur, but does not have a specific neuroanatomical basis. Intention tremors following severe head injuries, rarely associated with hydrocephalus and without other significant cerebellar findings, can develop as a dysfunction of the cerebellofugal outflow system. While chronic posttraumatic syndromes can be complex and difficult to treat, cerebellar stimulation has been utilized ipsilaterally to modulate limb spasticity, and bilateral ventrolateral cryothalamectomies staged 4-6 months apart have been successful in alleviating severe (intractable) intention tremors.
 ...
PMID:Rehabilitative neurosurgery: posttraumatic syndromes. 262 1

Dolichoectatic intracranial arteries (DIA) have been given several names, e.g. fusiform aneurysms, dolichomega-arteries. It is not an uncommon condition and it raises interesting points: 1) symptoms and signs result from compression (cranial nerve palsies; hydrocephalus) from ischemic or hemorrhagic episodes; 2) the diagnosis is made easier with CT since the arterial wall is often calcified; 3) the pathophysiology has long been assumed to be atherosclerotic in nature, but controversial assumptions are discussed; 4) associated arterial abnormalities are common: one of our cases and approximately half of the post mortem reported DIA were associated with aneurysm of the abdominal aorta. Two cases are reported here: 1) A 56 y/o male presented with progressive gait disorder, deafness and ischemic episodes. CT showed DIA and hydrocephalus. DIA involved the basilar artery, both intracranial carotid arteries, the abdominal aorta and probably one renal artery. A ventriculo-atrial shunt was performed unsuccessfully. Post mortem findings are reported; 2) A 52 y/o male had suffered from trigeminal neuralgia for about one year and from trigeminal permanent pain for two weeks. He had developed left hemiparesis and dysarthria. DIA involved the basilar artery and the left intracranial internal carotid artery. The right internal carotid artery and the right vertebral artery were hypoplastic. Clinical, radiographical and pathophysiological features are reviewed.
 ...
PMID:[Dolichoectatic intracranial arteries. Association with aneurysms of the abdominal aorta]. 305 65

A prospective long-term semiquantitative evaluation of the results of ventral intermediate-posterior ventral oral nucleus thalamotomy on the different aspects of dystonia was made in 29 patients with secondary disease, 12 with nonfamilial, eight with (non-Jewish) familial, and seven with atypical DMD. The effect of disease progression, even in secondary patients, on surgical outcome was reviewed. Thalamotomy resulted in a long-term improvement in limb function of more than 25% to 50% in 23% of the patients, over 50% in 34% of patients, but midline features responded poorly. Manual dexterity was little changed in secondary cases because of underlying paralysis but improved 38% in cases of DMD. Involvement of neck and trunk, of three to four limbs, and progressive disease prognosticated for a poorer result, but phasic and tonic, familial, and nonfamilial dystonia respond equally well and age at surgery made no difference. Significant complications in 29 secondary cases included one death 31 days postoperative, one case of worsened hemiparesis, two cases of worsened dysarthria, two cases of worsened locomotion, one case of hydrocephalus requiring shunting, and one case of need for permanent tracheotomy. In 27 cases of typical and atypical DMD, there were two instances of hemiparesis, two of significant speech deterioration, three of hand ataxia, one of postoperative seizures, and one of hydrocephalus requiring shunting for an overall significant morbidity rate of 21%. The limiting factor in treating secondary dystonia is the underlying spastic paralysis but that in DMD is the relentless postoperative progression. The overall results of this study are remarkably similar to those of other published series: a quarter of the patients improved by 25% to 50%, a quarter to a third by more than 50%. The analysis of effect on specific features of the disease may be useful in the future for predicting outcome in a particular patient.
 ...
PMID:Thalamotomy in generalized dystonia. 340 May 14

One week after chiropractic manipulations, a 60-year-old normotensive man suffered acute onset of vertigo, ataxia, and dysarthria. CT disclosed an isodense mass of the posterior fossa. Vertebral arteriography showed that the avascular mass arose from the right cerebellar hemisphere. Hydrocephalus developed, and suboccipital craniotomy revealed a large cerebellar hematoma due to a small arteriovenous malformation. A similar episode occurred 2 months later. CT isodense cerebellar hematomas should be considered before giving anticoagulant therapy.
 ...
PMID:Isodense cerebellar hematoma. 668 55

The natural history and the clinical and neuroimaging features of brainstem tumors in neurofibromatosis type 1 (NF1) are poorly understood. Magnetic resonance imaging (MRI) has been useful in NF1 in detecting intracranial abnormalities, especially of the brainstem. Brainstem tumors in NF1 have been confused clinically with non-NF1 brainstem tumors and radiographically with the increased T2 signal abnormalities, also known as "unidentified bright objects" (UBOs), which are common in NF1 and often located in the brainstem. This study, which evaluated 17 NF1 patients with brainstem tumors, is the largest series to date. Fifteen of 17 patients (88%) had neurologic signs and symptoms referable to brainstem dysfunction, including dysarthria, cranial neuropathies, and gross motor incoordination. Tumors were located primarily in the medulla in 14 of 17 NF1 patients (82%), in contrast to the pontine tumor location in the non-NF1 population. Seven NF1 patients (41%) required shunt placement for hydrocephalus at initial diagnosis, more frequent than in non-NF1 brainstem tumor patients. Six of 17 patients (35%) had evidence of radiographic tumor progression, but only three of them (18%) had correlative clinical progression. Two patients with progressive symptoms had partial surgical resection, and pathology revealed either fibrillary or anaplastic astrocytomas. Three patients were treated with radiation therapy, chemotherapy, or both, with two deaths. With a median follow-up of 52 months, 15 of 17 patients remain alive; 14 of them did not require adjuvant therapy. In our series, we describe NF1 brainstem tumors as a distinct clinical entity, much less aggressive than non-NF1 pontine tumors but more symptomatic than brainstem UBOs in NF1.
 ...
PMID:Brainstem tumors in patients with neurofibromatosis type 1: a distinct clinical entity. 747 89

"Cerebellar" mutism refers to a specific childhood disorder in which a complete but transient loss of speech, followed by dysarthria, occurs after removal of a cerebellar tumor. We present a consecutive series of 15 children with this disorder, which we prefer to designate "mutism and subsequent dysarthria." The conditions in which it develops suggest also an extracerebellar component of cerebellar mutism. Hydrocephalus at presentation, localization of tumor adjacent to the fourth ventricle, and postsurgical edema of the pontine tegmentum are involved in its development.
 ...
PMID:The syndrome of 'cerebellar' mutism and subsequent dysarthria. 796 50


1 2 3 4 Next >>