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)

Serial studies of auditory brainstem evoked responses (ABR) and slow vertex responses (SVR) were obtained during the progress of adrenoleukodystrophy in a 6-year-old boy. This child was normal until 5 years of age. His illness began with a gait disturbance, dysarthria, and hearing difficulty. Later, spastic paralysis, serious deafness, and blindness appeared. He died of respiratory failure 2 years after the onset. The ABR was normal at onset but changed to an abnormal pattern. Initially, there was lengthening of the wave V-I interpeak interval. This was followed by the disappearance of the later components as his general condition deteriorated. At the terminal stage, only a prolonged wave I was recordable. The postmortem pathology revealed demyelination of auditory nerves and remarkable neuronal loss in the auditory pathways of the brainstem; in addition, there was a variety of extensive degeneration throughout the cerebrum, in particular the complete degeneration of the white matter with secondarily occurring ganglionic cell changes. These date suggest that degeneration of the brainstem from rostral to caudal levels occurred.
 ...
PMID:The progress of adrenoleukodystrophy as revealed by auditory brainstem evoked responses and brainstem histology. 746 26

We report a 49-year-old man who presented progressive dysarthria, dysphagia, and left hemiparesis. The patient was well until June 28th of 1993 when he noted 'weakness' in his both legs; despite his weakness, he could play golf on that day. In the beginning of July, he noted difficulty in swallowing solid foods. He was admitted to the neurosurgery service of our hospital on July 15th of 1993 and a neurologic consultation was asked on July 17th. Neurologic examination at that time revealed an alert but somewhat childish man who appeared to have some difficulty in paying attention to questions. He was disoriented to time and showed difficulty in recent memory and calculation. Higher cerebral functions were intact. The optic fundi were normal; pupils were isocoric and reacted to light promptly; ocular movements were intact, however, he showed difficulty in convergence. Facial sensation and facial muscles were intact. He had no deafness. He showed slurred speech and difficulty in swallowing solid foods. The remaining cranial nerves were intact. Motor-wise, he was able to walk normally and no weakness or atrophy was noted. Mild ataxia was noted in the finger-to-nose and the heel-to-knee test on the left. Muscle stretch reflexes were normal and symmetric, however, the plantar response was extensor bilaterally. Sensation was intact and no meningeal signs were noted. General routine laboratory findings were unremarkable. CSF was under a normal pressure containing 1 cell/microliter, 68 mg/dl of protein, and 54 mg/dl of glucose. Cranial CT scan showed low density areas involving the pons, midbrain, left thalamus, and the left parietal cortex. In MRI, these areas presented low signal intensity in T1-weighted images and high signal intensity in T2-weighted in images. The brain stem appeared swollen. Gadolinium enhancement was negative. He was given a course of steroid pulse with 1 g/day of DIV methylprednisolone for three days followed by oral steroid. He showed only temporary improvement in swallowing. In the subsequent course, he showed progressive deterioration in dysarthria and dysphagia. A biopsy was performed on the left parietal lobe lesion. After biopsy, he was treated with steroid and glycerol without improvement. A course of chemotherapy with procarbazine, MCNU, and vincristine was given; he did not respond to chemotherapy. His left hemiparesis deteriorated. He developed aspiration pneumonia from dysphagia and expired on October 22, 1993. The patient was discussed in a neurologic CPC, and the chief discussant arrived at the conclusion that the patient had astrocytoma grade III involving the pons, midbrain, thalamus, and the parietal cortex.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:[A 49-year-old man with progressive dysarthria, dysphagia, and left hemiparesis]. 749 19

We report a 74-year-old man with a lung cancer, who developed right leg weakness, neurogenic bladder, and multiple cranial nerve palsies. The patient was well until December of 1992, when he was 74-year-old, when he noted transient double vision; in February of 1993, he noted numb sensation and weakness in his right leg. Later in the same month, he developed overflow incontinence of urine and weakness in his right face. He also noted deafness in his left ear (he had a marked loss of hearing in his right ear since childhood because of otitis media). His weakness in his right leg had progressed, and he was admitted to our service on March 19, 1993. On admission, he was afebrile and BP was 130/50 mmHg. General physical examination was unremarkable. On neurologic examination, he was alert and oriented to all spheres; no dementia was noted nor were detected aphasia, apraxia, and agnosia. His optic fundi were unremarkable; ocular movement appeared normal, however, he complained of diplopia in far vision. Sensation of the face was intact. He had right facial palsy of peripheral type; he was unable to close his right eye, and Bell's phenomenon was observed on attempted eye closure. On the left side, he had facial spasm. He had marked bilateral deafness. He had no dysarthria or dysphagia. The remaining of the cranial nerves were intact. Motor wise, he was unable to stand or walk alone; weakness did not appear to account for his difficulty in gait; manual muscle testing revealed 4/5 weakness in his tibialis anterior muscle, 1/5 in the peroneus longus, 0/5 in his extensor hallucis longus and extensor digitorum longus, all on the right side. Brachioradial and quadriceps femoris reflexes were increased to 3/4; plantar response was equivocal on the right side, and flexor on the left. Sensory examination revealed loss of touch and pain sensation in the L5 and S1 distributions in his right leg: vibration and position sensations were also diminished in his right foot. He had overflow urinary incontinence with loss of bladder sensation. Marked nuchal stiffness was noted, however, no Kernig's sign or eye ball tenderness was present. Pertinent laboratory findings were as allows; WBC 8,100/microliters, Ht 42.5%, platelet 326,000/microliters, TP 6.8 g/dl, BUN 16 mg/dl, creatinine 0.54 mg/dl, glucose 95 mg/dl, Na 136 mEq/l, K 4.4 mEq/l, Cl 100 mEq/l; liver profile was normal; CEA 436.6 ng/ml, CA19-93 U/ml; urinalysis was normal.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:[A 74-year-old man with urinary incontinence, right leg weakness and multiple cranial nerve palsies]. 766 22

Wolfram's syndrome is defined by the association of diabetes mellitus, diabetes insipidus, optic atrophy and nerve deafness. Other neurological anomalies, such as ataxia, nystagmus, tonic pupil, dizziness, dysarthria, dysphagia and epilepsy are rarely described and tend to appear later than the primary manifestations. We describe a patient with Wolfram's syndrome whose magnetic resonance image (MRI) of the head showed brainstem and cerebellar atrophy years before the appearance of clinical signs of brainstem disfunction. We conclude that alterations in MRI precede neurological symptoms by several years in Wolfram's syndrome.
 ...
PMID:[Wolfram's syndrome: correlation of clinical signs and neurological images]. 769 38

We report a 75-year-old woman with multiple cranial nerve palsies. The patient was well until January, 1992 when she had an onset of deafness in her left ear; she developed left facial pain in September, 1992, and came to the ENT clinic of our Izunagaoka Juntendo Hospital. She had chronic sinusitis; she was referred to neurology clinic on September 25 because of decrease in the superficial sensation in the second division of the left trigeminal nerve. She developed blurring of her left vision, and was admitted to the neurology service of Juntendo Izunagaoka Hospital on December 7th, 1992. On admission, general physical examination was unremarkable. Neurologic examination revealed alert and mentally sound woman; higher cerebral functions were intact. In the cranial nerves, olfactory sensation was normal; the left vision was reduced to discriminate light and dark; the right vision was normal. Pupils were round and isocoric, but the light reflex was sluggish on the left side; the abduction of the left eye was impossible; other ocular muscles appeared intact. Sensation of the left face was almost completely lost; the corneal reflex was lost on the left side; no jaw deviation was noted. She had near complete left facial palsy of the peripheral type; the left ear was deaf. The movement of the left soft palate was slightly weak, but no deviation of the uvula was noted; she had no dysarthria or dysphagia.2/
 ...
PMID:[A 75-year-old woman with multiple cranial nerve palsies and a paranasal mass]. 806 43

Language disturbances in cerebral palsy with mutism. The analysis of the afferent side of language in 18 cerebral palsy children with no oral expression shows that they form a very heterogeneous population. The semiological analysis concerning comprehension leads to their classification into four main groups akin to the classical dysphasia syndromes. Major agrammatism occurring in phonologico-syntactic dysphasia. Sensory dysphasias (or verbal deafness). Mixed (or global) dysphasias. Motor dysphasias: phonological programmation disturbances (anarthria) and motor production disturbances (dysarthria). The disclosure of these various dysphasias allows for specific rehabilitation and for education procedures suitable for each individual patient.
 ...
PMID:[Symptomatology of language disorders in cerebral palsy with mutism]. 821 52

Wolfram's syndrome, also known as DIDMOAD syndrome, includes juvenile diabetes mellitus and optic atrophy variously associated with diabetes insipidus and deafness. We describe the neurological findings in 5 patients with Wolfram's syndrome. All patients had a neurological examination and were subjected electrophysiological and brain imaging including CT scan and, in one patient, MRI. There were two pairs of brothers and a sporadic case with paternal consanguinity suggesting recessive inheritance. Neurological abnormalities were found in four patients including dysarthria, seizures, anosmia, nystagmus, ataxia and changes in the electroencephalograms, electroretinograms and evoked potentials. In contrast with previous reports, four patients had abnormal brain CT scan with prominent atrophy of the brainstem. In the patient studied with NMR, severe brainstem and cerebellar atrophy was found. These neuroradiological findings are reminiscent of those described in olivopontocerebellar atrophy and are in agreement with previous pathological studies. We conclude that Wolfram's syndrome includes phenotypical manifestations of olivopontocerebellar atrophy. This reinforces the opinion that olivopontocerebellar atrophy is a nonspecific syndrome of varied causes.
 ...
PMID:[Neurologic manifestations in Wolfram's syndrome]. 833 58

We report a 24-year-old man who presented unilateral multiple cranial nerve involvements followed by progressive paraplegia. The patient expired after developing DIC and pneumonia. Post-mortem examination revealed Ewing's sarcoma originated in the pubic bone with extensive metastases including the clivus which was responsible for his cranial nerve lesions. The patient was well until 24 years of age when he noted an onset of pain and a mass in the pubic region. The histology of the biopsy specimen of the tumor suggested Ewing's sarcoma. He was treated with chemotherapy and local radiation. A year after, he noted an onset of nuchal pain, difficulty in tongue movement, dysarthria, deafness in the left ear, and diplopia. On admission to our hospital in July 1990, neurological examination revealed an alert and intelligent Japanese male in no acute distress. The olfactory to the trigeminal nerves appeared intact. He showed complete abducens nerve palsy, facial weakness, mild deafness, and weakness of the soft palate, the sternocleidomastoid muscle and the tongue, all on the left side. The remainder of the neurological examination was unremarkable except for dysesthesia along the left C8 and Th1 dermatoms. Radiological examination revealed a 10 x 10 cm sclerotic mass in the public bone and a high signal mass lesion between the clivus and the pons in the T2-weighted MRI. His clinical course was complicated by acute paraplegia with anesthesia below the Th4 dermatom, DIC, and respiratory distress due to plural effusion. Post-mortem examination revealed a necrotic and hemorrhagic tumor in the pubic bone. The histology was consistent with Ewing's sarcoma.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[A 24-year-old man presenting Garcin syndrome and paraplegia]. 847 71

Two cases (case 1, a 45-year-old man; case 2, a 68-year-old man) of superficial siderosis of the central nervous system are presented. Main neurological symptoms were anosmia, sensorineural deafness, dysarthria, ataxia, and pyramidal tract signs. Lumbar puncture revealed bloody cerebrospinal fluid (CSF) in both cases. In case 1, the CSF became watery clear after administration of hemostatic medicines. T2-weighted magnetic resonance images showed cerebellar atrophy and marginal hypointensity of the brainstem, cerebellum, and the entire spinal cord. T2-weighted images of the cranial nerves showed hypointensity of the VIII nerves which were clinically impaired as compared with normointensity of the VII nerves which presented no clinical symptom. These findings may reflect difference in the degree of hemosiderin depostion between the VII and VIII nerves. While case 1 had a borderline score of WAIS-R (IQ79), case 2 showed overt dementia (performance IQ65). Positron emission tomography showed that cerebral blood flow and cerebral oxygen metabolism were reduced in the basal temporal lobes in both cases.
 ...
PMID:[Two cases of superficial siderosis of the central nervous system. Findings of the cerebrospinal fluid, magnetic resonance imaging and positron emission tomography]. 882 97

Hearing impairment is a rare but characteristic symptom of vertebrobasilar occlusive disease. Two patients with anterior inferior cerebellar artery (AICA) infarction and hearing loss as presenting complaint, are described. In patient 1 progressive bilateral AICA infarction was caused by occlusion of the basilar artery, verified by transcranial Doppler sonography. Initial dizziness and bilateral hearing loss were followed by ataxia, dysarthria, dysphagia and right facial sensory impairment. Hearing loss improved during the second week, parallel to a temporary partial recanalisation of the basilar artery, but the patient subsequently developed further progressive brain stem symptoms and died. A right-sided AICA-infarction was diagnosed in patient 2. Initial symptoms were right-sided deafness and dizziness. Neurological examination revealed nystagmus, ataxia and involvement of th 5th and 6th cranial nerve. A progressive improvement of hearing loss and total recovery of the other brain stem symptoms was evident. In conclusion every patient with sudden hearing loss should be examined for additional brain stem symptoms since this can be the presenting sign of a life-threatening basilar artery thrombosis.
 ...
PMID:[Hearing loss as the leading symptom in anterior inferior cerebellar artery infarction]. 956 60


<< Previous 1 2 3 4 5 Next >>