Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is well recognized that food contamination can result in botulism either from ingestion of performed toxin, in classical botulism, or through absorption of toxin from bacteria within the gut, in infant botulism. Botulism due to contamination of wounds with Clostridium botulinum is not commonly recognized. We report a case of wound botulism occurring in an eight-year-old boy, characterized by early ptosis, dysphagia and dysarthria and then followed by progressive generalized paralysis and fixed dilated pupils, but with intact sensorium. Management consisted of early wound debridement and prolonged intensive respiratory and nutritional support. Recovery was complete.
 ...
PMID:Wound botulism. 742 26

A surgically treated case of ventrally exophytic pontine glioma is reported. A 49-year-old woman, complaining of dysarthria, dysphagia and gait disturbance, was admitted to our department. Her past history included bronchial asthma. Plain skull x-p and tomography revealed destruction of the dorsum sellae and upper clivus. CT demonstrated an enhanced oval mass at the ventral side of the upper brainstem. The mass was severely compressing the brainstem dorsally. MRI revealed a low-intensity band between the tumor and the brainstem. Dynamic MRI demonstrated a pattern of rapid increasing and slow reduction. Cerebral angiogram demonstrated a paradoxical sign that pontine branches were located anterior to the basilar artery. The finding demonstrated that the tumor was an intraaxial mass. The first operation was performed by the orbitofrontomalar approach. On the trans-sylvian route, the tumor was removed partially with CUSA and neuronavigation system. Its histology was astrocytoma grade III. Radiation therapy was added. The patient's symptoms aggravated again. On the second operation, the transtemporal route with tentorial resection was chosen. Under a wide visual field, intracapsular subtotal resection of the tumor was performed. Interferon therapy was added. She was discharged to her home with no neurological deficits. Ventrally exophytic pontine glioma is very rare. Low-intensity band of MRI, a sign of extraaxial mass, was visualised in our case. On the other hand, cerebral angiogram demonstrated a paradoxical sign. This sign suggested that the tumor originated from the brainstem. With update skull base surgery and neuronavigation system, surgical therapy of ventrally exophytic pontine glioma is safe and effective.
 ...
PMID:[A surgically treated case of ventrally exophytic pontine glioma]. 747 14

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 describe a family with autosomal dominant progressive supranuclear palsy (PSP) involving five generations which was confirmed in one patient. The proband presented with progressive slowness at age 53 years, followed by ocular palsy, loss of balance, axial dystonia, dysphagia and dysarthria, and died at age 59 years. Neuropathological examination revealed moderate numbers of neurofibrillary tangles without prominent senile plaques in the cortex, and neuronal loss, gliosis and moderate to severe accumulation of tangles in the basal ganglia and brainstem. Other affected relatives, including the proband's sister, father, paternal uncle, and other members of earlier generations presented with non-characteristic akinetic syndromes, which progressed towards more typical PSP only after several years of disease. A review of the literature revealed six other families with neurodegenerative disorders associated with pathological findings compatible with PSP in at least one member. The clinical symptoms varied greatly between individuals in these families. The pattern of inheritance seems compatible with autosomal dominant transmission, although other patterns of transmission could not be excluded. We conclude that there is an autosomal dominant form of PSP and that the number of hereditary cases may be greater than previously thought. The rarity of familial cases of PSP could be attributed to diagnostic problems, including lack of recognition of atypical cases and death of the gene carriers before the age of appearance of the clinical symptoms. Large families with hereditary PSP could provide an adequate point of departure for investigation of the gene defect responsible for this disease.
 ...
PMID:Familial progressive supranuclear palsy. Description of a pedigree and review of the literature. 749 73

We attempted to correlate the marked neurological asymmetry observed in two amyotrophic lateral sclerosis patients with their histopathological lesions. Patient 1, a 52-year-old man, developed dysarthria and dysphagia, followed by muscle weakness in the left arm and then of the left leg. Patient 2, a 44-year-old man, developed muscle weakness in the left hand, left leg, tongue with left-sided predominance, right hand and right leg in that order of progression. Both patients exhibited moderate to marked left-sided predominant involvement of the lower motor neuron system, accompanied by retained or hyperactive deep tendon reflexes on the left side in the early stage of their illness. Most of the asymmetry in the lower motor neuron system involvement persisted until the death of the patients. Histopathological examinations, including semiquantitative analysis, revealed that both patients exhibited left-sided predominant degeneration of the lower motor neuron system at those spinal cord levels where the neurological asymmetry was of a moderate to marked degree. In addition left-sided predominant degeneration of the lateral corticospinal tracts was seen in both patients and right-sided predominant involvement of Betz cells in the leg area of the motor cortex of patient 1. This pattern of both the neurological and histopathological asymmetry suggested the probable existence of an intimate somatotopically related linkage between the upper motor neuron system degeneration and lower motor neuron system degeneration in both patients.
 ...
PMID:Amyotrophic lateral sclerosis with marked neurological asymmetry: clinicopathological study. 757 78

We compared locations of infarctions and clinical characteristics for patients with dysarthria and those without dysarthria. Subjects were 40 patients with a small infarction in the corona radiata or junctional zone to the capsule and 13 patients with infarctions in the internal capsule. Left corona radiata/junctional zone infarctions were significantly smaller than right sided lesions. Dysarthria was associated more frequently with the corona/junctional lesions on the left side than the right sided lesions. Asymptomatic infarctions on the contralateral side were seen in 41% of the patients with dysarthria. In these cases, dysarthria continued longer and dysphagia occurred more frequently than the cases without right sided lesions. Corona radiata/junctional zone infarctions with dysarthria were located significantly more anteriorly than those without dysarthria. The corona radiata/junctional zone infarctions presenting with dysarthria alone, upper limb dominant hemiparesis, and lower dominant hemiparesis were located in the anterior, middle, and posterior areas, respectively. In conclusion, dysarthria may occur with unilateral small cerebral infarctions, more frequently with left sided lesions than with right sided lesions. It is assumed that the left corona radiata/junctional zone infarction may interrupt simultaneously the corticobulbar pathway and callosal fibers to the right hemisphere which transmit motor information for speech to the right hemisphere. It is also possible that there are individual variations in the proportion of crossed and uncrossed corticobulbar innervation, which may explain dysarthria with unilateral cerebral lesions in some patients. It was suggested that there is an anterior-posterior somatotopy in the corona radiata/junctional zone as well as in the internal capsule.
 ...
PMID:[Dysarthria due to small cerebral infarction--the localization of lesion and clinical characteristics]. 761 59

Striatonigral degeneration (SND) is difficult to diagnose in vivo. The purpose of this study was to detect the best indicators for an early and reliable diagnosis of the disease. Eighteen patients clinically diagnosed as having SND were selected with rigorous inclusion criteria and compared to 18 patients with Parkinson's disease (PD) matched for age and disease duration. Apart from dysautonomia, the principal discriminant clinical features that distinguished SND from PD were the early appearance of the following symptoms and signs: (a) severe and atypical progressive parkinsonism characterized by bilateral bradykinesia and rigidity, slowness of gait, postural instability, and falls, and poor or absent response to adequate levodopa treatment; (b) increased tendon reflexes associated or not with frank pyramidal signs, severe dysarthria, and less consistently, dysphagia, stridor, antecollis, and stimulus-sensitive myoclonus, which, when present, are highly suggestive of the disease.
 ...
PMID:"Pure" striatonigral degeneration and Parkinson's disease: a comparative clinical study. 765 45

We report a 54-year-old man with progressive proximal muscle atrophy and gynecomastia. The patient had an insidious onset of weakness in his lower extremities at age 14, in that he noted a difficulty in standing up from a chair. Soon after he noted some difficulty in climbing up stairs. At age 35, he noted weakness in his arms; his weakness slowly progressed in that he became unable to walk or stand alone before 40 years of age. He also noted gynecomastia at that age. He was admitted to our hospital for the work up on September 16, 1993, when he was 54-year-old. On admission, he was alert and oriented; his BP was 150/70 mmHg; he had bilateral gynecomastia, however, no other skeletal deformities were found. On neurologic examination, he was mentally sound without dementia, and his higher cerebral functions were normal. Cranial nerves also appeared intact without facial atrophy, dysarthria, or dysphagia; no atrophy was noted in the tongue. He had marked muscle atrophy in both upper and lower extremities more marked in the proximal portions; muscle strength was approximately in the range of 2/5 to 3/5 in the proximal parts, and 4/5 in the distal parts in both upper and lower extremities. No fasciculation was noted; muscle tone was flaccid; no ataxia was present. Deep reflexes were either lost or markedly diminished. No Babinski sign was noted. Sensation was intact. Laboratory examination revealed normal blood counts; serum CK was slightly increased to 131 IU/l; ECG showed complete right bundle branch block; EMG revealed no active units in the right biceps brachii, deltoid, quadriceps femoris, and triceps surae muscles; in other muscles tested, motor unit potentials of low amplitude and short duration were seen; in the right tibialis anterior muscle, however, motor unit potentials with an amplitude up to 6 m V were also seen. Nerve conduction velocities were normal. A diagnostic procedure was performed. He was discussed in the neurological CPC, and the chief discussant arrived at the conclusion that this patient had Becker type of progressive muscular dystrophy. In her differential diagnosis, the possibility of Kennedy-Alter-Sung syndrome was discussed because this patient had gynecomastia. However, the discussant excluded that possibility because of absence of both bulbar symptoms and typical neurogenic changes in his EMG. The diagnostic procedure was a muscle biopsy on the left tibialis anterior muscle. Histologic observation on HE stained specimens revealed marked inequality in the muscle fiber diameters, increase in endomysial nuclei, proliferation of connective tissue, and fiber splitting.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:[A 54-year-old man with progressive proximal muscle atrophy and gynecomastia]. 766 8

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


<< Previous 1 2 3 4 5 6 7 8 9 10