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

We sought clinical and radiological findings of 150 consecutive patients with acute isolated pontine infarct who were admitted to our Stroke Unit over 6 years. In all patients CT, MRI and magnetic resonance angiography (MRA) were performed during the hospitalization. On clinico-radiological analysis regarding the pontine lesion boundaries there were five main clinical patterns that depended on the constant territories of intrinsic pontine arteries: (1). anteromedial pontine syndrome (58%) presented with motor deficit with dysarthria, ataxia, and mild tegmental signs in one third of patients; (2). anterolateral pontine syndrome (17%) developed with motor and sensory deficits in half of the patients, and were associated with tegmental signs (56%) more frequently than the anteromedial infarct syndrome; (3). tegmental pontine syndrome (10%) presented with mild motor deficits and associated with sensory syndromes, eye movement disorders and vestibular system symptoms including vertigo, dizziness and ataxia; (4). bilateral pontine syndrome (11%) consisted with transient consciousness loss, tetraparesis and acute pseudobulbar palsy; (5). unilateral multiple pontine infarcts (4%) were rarely observed, and were always associated with severe sensory-motor deficits and tegmental signs. In our series, there was no infarct in the extreme dorsal and lateral tegmental pontine territories which have been mostly associated with cerebellar infarctions. The main etiology of stroke was basilar artery branch disease (BABD) in 59 patients (39%), followed by small-artery disease (SAD) in 31 (21%), large-artery disease of vertebrobasilar arteries in 27 patients (18%), cardioembolism in 12 (8%) and in 16 patients (11%) no cause of stroke was found. Our findings suggest that it is possible to identify clinical subgroups of pontine infarction, in which BABD and SAD were the most common causes of stroke. After an acute onset, outcome is in general excellent except in those with bilateral pontine lesions.
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PMID:Clinical spectrum of pontine infarction. Clinical-MRI correlations. 1252 87

Seizures are an uncommon but serious complication of hyponatremia which can lead to permanent brain damage and even death. It is recommended that patients with hyponatremic-induced seizures be treated with 3% hypertonic saline, however, a rapid rate of correction may result in central pontine myelinolysis (CPM), a severe neurological disorder characterized by mutism, dysarthria, spastic quadriparesis, and pseudobulbar palsy. The patient in this case developed a hyponatremia-induced generalized tonic-clonic seizure which was aborted by rapid therapy with diazepam, followed by hypertonic saline and phenytoin. Subsequent replacement of hypertonic saline with normal saline and salt tabs in combination with phenytoin allowed gradual correction of serum sodium without any subsequent seizures or neurological complications.
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PMID:Therapy with hypertonic saline in combination with anti-convulsants for hyponatremia-induced seizure: a case report and review of the literature. 1263 26

Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder characterised by pseudobulbar palsy, cognitive deficits and epilepsy associated with bilateral perisylvian cortical dysplasia on neuroimaging studies. We report a long-term follow-up of a 18-years girl diagnosed with CBPS according to the typical clinical and magnetic resonance imaging (MRI) features. The patient showed faciopharyngoglossomasticatory diplegia, severe dysarthria, ataxia, spastic quadriparesis and severe mental retardation. Brain MRI evidenced bilateral perisylvian cortical dysplasia. Since early life she suffered from complex febrile seizures and epilepsy consisting of complex partial attacks with affective manifestations associated with centro-temporal EEG abnormalities. During 18 years of follow-up she was treated with phenobarbital, carbamazepine, lamotrigine, gabapentin but did not show any significant clinical improvement. Subsequently, monotherapy with phenytoin (PHT) was followed by a significant clinical improvement. At age 17, because of adverse effects, PHT was gradually substituted by topiramate (TPM). Full control of seizures was obtained at the age of 17 years with TPM. EEG abnormalities throughout the years have been reduced according to the clinical course. These findings emphasised the importance of long-term follow-up, suggesting that the prognosis for epilepsy may not be predicted based on the early response to treatment or on the presence of structural encephalic abnormalities, as reported in the literature.
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PMID:Congenital bilateral perisylvian syndrome with partial epilepsy. Case report with long-term follow-up. 1562 42

Structural abnormalities related with pseudobulbar palsy have been gaining attention because of their characteristic symptoms and unique pathogenesis. We present five cases of bilateral perisylvian ulegyria (BPU) presenting epilepsy and pseudobulbar palsy with pathogenesis different from previously reported syndromes. All patients showed medically intractable seizures, complex partial seizures with secondary generalization and clinical symptoms of pseudobulbar palsy, including dysarthria, limitation of tongue movement and drooling. MRI revealed BPU in all patients, and BPU associated with hippocampal sclerosis in four patients. Intracranial EEG recording with subdural grip and stripe was helpful for localizing the area of ictal generation. Resective surgeries, including the temporal lobe, central area and parietal lobe, were performed depending on the localizing information. The surgical outcome was favorable after 9.8 years of follow-up. Characteristic features of ulegyria were confirmed on pathological examination. Ulegyria is considered to be another important perinatal or postnatal structural abnormality which can explain the etiological heterogeneity for pseudobulbar palsy, which results from bilateral perisylvian lesions. Awareness of this disorder can provide a useful strategy for evaluation and treatment which differs from that in perisylvian polymicrogyria.
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PMID:Bilateral perisylvian ulegyria: clinicopathological study of patients presenting with pseudobulbar palsy and epilepsy. 1677 Nov 81

We report herein a 61-year-old man with diffuse leukoencephalopathy, subcortical infarcts and cervical and lumbar spondylosis. Medical history included baldness and lumbar spondylosis at young-adult onset. His parents were consanguineous (cousin). He had been experiencing severe lumbago since 20-years-old, with hair loss starting around the same time. He noticed dysarthria and gait disturbance at 59-years-old. He was admitted to our hospital at 61-years-old with aggravation of gait disturbance. On admission, no abnormalities were evident on physical examination except for diffuse baldness. Neurological findings included mild dementia, bilateral hyperreflexia, paraparesis, right Babinski's sign, and pseudobulbar palsy. Blood pressure was normal. T2-weighted imaging of the brain revealed diffuse high-intensity in the periventricular white matter and subcortical infarcts in the brainstem and bilateral basal ganglia. Marked lumbar deformations were observed on spinal MRI. Clinical features in this case met the criteria for cerebral autosomal recessive arteriopathy with subcortical infarctions and leukoencephalopathy (CARASIL), apart from late onset of cerebral infarction.
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PMID:[Late-onset leukoencephalopathy without hypertension in a case of young-adult-onset alopecia and spondylosis: a variant of CARASIL?]. 1861 51

Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are osmotic demyelination syndrome. A 45-year-old man with a history of alcoholism visited the ER with dysarthria and dysphagia for 2 days. These symptoms occurred 3 days after he had stopped drinking alcohol. The neurological symptoms progressed to anarthria, pseudobulbar palsy and gait disturbance. During admission, the electrolyte studies were within the normal range. Diffusion-weighted images revealed high signal intensities in the pons, thalamus and basal ganglia. Apparent diffusion coefficient image showed low signal intensities in the pontine lesion, but isosignal intensities in the extrapontine lesion. The symptoms gradually improved after 1 month with only conservative treatment. The 1 month-follow-up MRI showed significant reduction of the previous extrapontine lesions. These findings suggest that cytotoxic edema is central to the pathogenesis of CPM, but vasogenic edema plays an important role in the pathogenesis of EPM occurring during alcohol withdrawal.
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PMID:Central pontine and extrapontine myelinolysis that developed during alcohol withdrawal, without hyponatremia, in a chronic alcoholic. 2022 3

We describe clinically mild encephalopathy with a reversible lesion in the splenium of the corpus callosum associated with the novel swine-origin influenza A (H1N1) virus. A 14-year-old Japanese boy was hospitalized because of dysarthria and dysphagia 5 days after the onset of fever. He had been receiving zanamivir for 4 days before admission. Diffusion-weighted magnetic resonance imaging on clinical day 6 revealed lesions in the splenium of the corpus callosum and bilateral frontoparietal white matter. With continued zanamivir treatment, his signs completely resolved within 24 hours, and the abnormal radiologic signals resolved 3 days later. Neurologic signs were limited to pseudobulbar palsy, without impairment of consciousness or seizures. This presentation is, to our knowledge, the first among patients with mild encephalopathy with a reversible lesion in the splenium of the corpus callosum, expanding the clinical spectrum of this condition. Our findings indicate that pandemic 2009 influenza A (H1N1) infection can cause mild encephalopathy with a reversible lesion in the splenium of the corpus callosum.
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PMID:Reversible splenial lesion associated with novel influenza A (H1N1) viral infection. 2047 1

Slowly progressive dysarthria over many years may be the only sign of primary lateral sclerosis (PLS). Clinically it presents as pseudobulbar palsy which can be differentiated from amyotrophic lateral sclerosis (ALS) by the longer disease duration (> or =4 years), central pathological magnetic-evoked potentials to the tongue and lack of denervation in EMG. In contrast, hereditary spastic paraplegia (HSP) is characterized by a primary spasticity of the lower limbs, mostly later onset, the fact that other family members are affected and in isolated cases by positive genetic testing for mutations.
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PMID:[Slowly progressive dysarthria in primary lateral sclerosis]. 2053 74

We report a 59-year-old immunocompetent man presenting with slowly progressive gait unsteadiness, dysarthria, and clumsiness in writing over 6 months. There were bilateral pyramidal signs, pseudobulbar palsy, and attention deficits. Cerebrospinal fluid examination showed mild mononuclear pleocytosis, and magnetic resonance imaging revealed pachymeningeal pattern of contrast enhancement beneath the calvarium and the posterior cranial fossa. Interferon-gamma release assay in whole blood after stimulation by specific tuberculosis antigens was positive and repeat polymerase chain reaction assay detected Mycobacterium tuberculosis genome in the cerebrospinal fluid. After combination therapy with anti-tuberculous agents and corticosteroids, the patient's pachymeningitis regressed. Tuberculous cranial pachymeningitis may present with chronic diffuse brain dysfunction without headache, fever, or cranial nerve dysfunction.
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PMID:[Tuberculous cranial pachymeningitis presenting with long-standing diffuse brain dysfunction]. 2159 96

We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis.
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PMID:Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia. 2357 Sep 81


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