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 reported a patient with bilateral cerebellar peduncle infarcts who had an abrupt onset of bilateral hearing loss. A hypertensive 56-year-old man suddenly experienced bilateral hearing loss without other accompanying neurological deficits. He was hospitalized and treated for "idiopathic deafness". In addition, dysarthria and ataxic gait appeared two days later and he was transferred to our hospital. On neurological examination, the patient presented with diplopia, neurosensory hearing loss (approximately 70 dB) ataxic dysarthria, bilateral cerebellar ataxia and bilateral Babinski's signs. Auditory brain stem evoked response demonstrated prolonged delay of interpeak latency between waves III-IV. CT and MRI revealed fresh ischemic lesions symmetrically located at the middle cerebellar peduncles and cerebellar medullary body. Cerebral angiography showed total occlusion of the left vertebral artery and a stenotic right vertebral artery at the ostium of the posterior inferior cerebellar artery. We postulated that hearing impairment in this patient resulted from transient ischemia of the bilateral auditory tract in the brain stem or the peripheral cochlear system, but the definitive cause of the transient hearing loss remains undetermined. Concomitant appearance of a symmetrical infarction at the cerebellar peduncles is rare. We suggest that a circulation defect involving a multivascular system, which resulted in "border zone infarction" occurred at these regions.
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PMID:[A case of bilateral cerebellar peduncle infarction with bilateral hearing impairment of a sudden onset]. 795 15

Mild overdosage of phenytoin produces reversible cerebellar symptoms (nystagmus, double vision, dysarthria and ataxia). Several case reports suggest that relatively mild and relatively short intoxication can lead to cerebellar degeneration. We observed 11 patients who had episodes of abnormally increased serum levels, most of which developed clinical signs of cerebellar dysfunction. All of these patients were examined with a 1.5-tesla whole-body system (Magnetom, Siemens). Five patients had normal cerebellar structures, although 3 of them had a history of clinical intoxication and all had at least one episode of increased serum level of diphenylhydantoin. The remaining 5 had moderate to severe cerebellar atrophy. Two of them never experienced signs of clinical intoxication. There was no correlation between degree of atrophy and severity of clinical symptoms and elevation of serum DPH levels. There was no correlation between cerebellar atrophy, duration of epilepsy and frequency of seizures.
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PMID:Phenytoin overdosage and cerebellar atrophy in epileptic patients: clinical and MRI findings. 800 16

We report the fourth case of cerebral demyelinating disease associated with 5-fluorouracil and levamisole hydrochloride therapy for adenocarcinoma of the colon. The initial manifestations included subacute progressive decline in mental status, ataxia, dysarthria, and diplopia. Magnetic resonance imaging of the head demonstrated multifocal enhanced lesions in the white matter. The patient experienced improvement, without corticosteroid treatment, from 2 weeks to 4 months after cessation of chemotherapy.
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PMID:Multifocal leukoencephalopathy: occurrence during 5-fluorouracil and levamisole therapy and resolution after discontinuation of chemotherapy. 845 95

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)
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PMID:[A 24-year-old man presenting Garcin syndrome and paraplegia]. 847 71

Listeria monocytogenes rhombencephalitis has never been studied in a significant group of patients. We describe 14 adult cases who were seen over a 10-year period. A biphasic illness was characteristic: (1) prodromes (5-15 days) with malaise, fatigue, headache, nausea or vomiting, and fever; (2) cranial nerve palsy with facial palsy, diplopia, dysphagia, dysarthria, usually multiple. Meningism and hemi- or tetraparesis were present in 11 patients and cerebellar dysfunction in 9 patients. In 4 cases, CT showed widening of the brain stem with disappearance of the surrounding cisterns. The cerebrospinal fluid was abnormal in all patients in whom this investigation was done (pleocytosis, elevation in protein content). The patients received antibiotic therapy for 2-6 weeks. In the 9 patients who recovered, the neurological dysfunction improved within 2 days to 1 week of the initiation of therapy. There were 5 deaths. At autopsy in 2 cases, there was severe purulent meningitis and rhombencephalitis with predominantly polymorphonuclear cellular infiltration in 1 case, while numerous microabscesses in the midbrain, pons and medulla were observed in the other. We conclude that L. monocytogenes infection should be considered in patients who develop fever and focal neurological signs particularly localized to the brain stem.
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PMID:Early symptoms and outcome of Listeria monocytogenes rhombencephalitis: 14 adult cases. 849 12

Among 17 patients with amyloid polyneuropathy type IV in a Japanese family, we found a 72-year-old woman, who showed extraocular symptoms approximately 10 years after the onset of the disease. she developed weakness of the right facial muscles and dysarthria at age 57. She had atrophy and disturbance of movement of the tongue, along with difficulty in swallowing at age 62. At the age of 66, she felt diplopia when she looked toward the left, followed by difficulty of ocular movement. These manifestations progressed and at age 72, she was found to have mild ptopsis, mild to moderate disturbance of almost all extraocular muscles, moderate to severe disturbance of facial, masseter, pharyngeal, tongue and neck muscles. She also had slight weakness and atrophy of the limb and truncal muscles together with slight loss of pain and vibratory sensations in the distal parts of the limbs. FAP IV has sometimes been called cranial amyloidosis, but motor disturbance is limited to the middle and lower cranial nerve territories in the majority of the reported cases, and manifestations of the extraocular muscles are quite rare. According to the present investigation of the world literature, this is the second case of FAP IV with extraocular muscle involvement.
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PMID:[Familial amyloidosis of the Finnish type (FAP) with extraocular muscle involvement]. 856 42

Fabry's disease (FD) is a rare, sex-linked disorder resulting from alpha-galactosidase deficiency. Cerebrovascular complications have been reported in the literature but have not been systematically analyzed. We report 2 patients and review 51 previously reported cases (descriptive meta-analysis) to clarify the clinical, radiologic, and pathologic features. The average age at onset of cerebrovascular symptoms was 33.8 years for hemizygous individuals (n = 43) and 40.3 years of heterozygotes (n = 10). The most frequent symptoms and signs were as follows (in descending order of frequency): hemiparesis, vertigo/dizziness, diplopia, dysarthria, nystagmus, nausea/vomiting, head pain, hemiataxia, and ataxia of gait, in the hemizygote group; and memory loss, dizziness, ataxia, hemiparesis, loss of consciousness and hemisensory symptoms, in the heterozygote group. The vertebrobasilar circulation was symptomatic in 67% of the hemizygotes and 60% of the heterozygotes. Intracerebral hemorrhage was found in 4 patients (3 hemizygotes and 1 heterozygote). Elongated, ectatic, tortuous vertebral and basilar arteries were the most common angiographic and pathologic features. For the hemizygotes, the recurrence rate for cerebrovascular disease was 76% and the death rate was 55%; 86% of the heterozygotes had recurrent cerebrovascular event(s) and 40% died. The cerebrovascular manifestations of FD, in both hemizygotes and heterozygotes, are predominantly due to dilative arteriopathy of the vertebrobasilar circulation, frequently recur, and portend a poor prognosis.
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PMID:Cerebrovascular complications of Fabry's disease. 868 96

A 39 year-old severely disabled woman with a 19 year history of chronic relapsing-remitting multiple sclerosis (MS) began to experience improvement in symptoms within 24 hours after she received experimental treatment with picotesla electromagnetic fields (EMFs). Pattern reversal visual evoked potential (VEP) study obtained three weeks after the initiation of the first magnetic treatment showed a return to normal of the P100 latencies in each eye. The patient continued to receive 1-2 EMFs treatments per week and during the following 32 months she made a dramatic recovery with resolution of diplopia, blurring of vision, dysarthria, ataxia of gait, and bladder dysfunction as well as improvement in fatigue, heat tolerance, mood, sleep, libido, and cognitive functions. VEP studies, which were repeated in April of 1995 more than 2 1/2 years after the initiation of magnetic treatment, showed that P100 latencies remained normal in each eye providing objective documentation that continued application of these EMFs may sustain normal conduction in the damaged optic pathways over a long period of time. This is the first case report documenting the dramatic long term beneficial effects of treatment with picotesla range EMFs in a patient with MS.
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PMID:Long term beneficial effects of weak electromagnetic fields in multiple sclerosis. 874 48

Syringobulbia is an uncommon lesion of the central nervous system. It is defined as a pathological cavitation of the brain stem. The most common symptoms are headache, vertigo, dysphonia or dysarthria, trigeminal paraesthesia, dysphagia, diplopia, tinnitus, palatal palsy. Syringobulbia occurs with atlantoaxial congenital abnormalities (Chiari malformation), infection, tumours, and other causes. The idiopathic syringobulbia is however a rare finding. Early surgical treatment is the treatment of choice. We report on a 58-year old female patient with idiopathic syringobulbia. She complained of occipital headaches and vertigo. On examination she had horizontal nystagmus and diplopia. Occipital headaches and vertigo were improved after operation. We review the literature on syringobulbia, and discuss the clinical features of this uncommon condition.
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PMID:[Isolated idiopathic syringobulbia: case report and summary of the literature]. 877 68

A 28-year-old man was admitted to our hospital, because of double vision, memory disturbance and dysarthria. These symptoms developed in November, 1994. His mental activity was gradually decreased and he became apathetic. A physical examination on admission was unremarkable. There were no lymphadenopathy, hepatosplenomegaly or skin rash. Neurological examination revealed disorientation, decreased mental activity and left gaze-evoked nystagmus. Neuropsychological tests demonstrated impairment of recent memory and performance but frontal lobe functions were maintained. Laboratory data showed hyponatremia, elevation of antinuclear antibodies and IgG-antibodies to Epstein-Barr virus capsid antigen and Epstein-Barr virus nuclear antigen. A brain MRI revealed multiple lesions in the cerebral subcortex, cerebellum and brainstem. These lesions showed high signal intensity on T2-weighted images and enhanced high signal intensity on T1-weighted images using Gd-DTPA injection. A cystic mass was seen between bilateral lateral ventricles and contacted with the fornix and thalamus. Lymphomatoid granulomatosis was diagnosed by an open brain biopsy, which showed diffuse infiltration of small lymphocytes, histiocytes and plasma cells, especially around the small vessels. Epithelioid granuloma or necrosis was not seen. An immunohistochemical study showed that nearly all lymphocytes were MT-1 positive T lymphocytes. The patient was treated by whole brain irradiation (30Gy) and pulse therapy of methylprednisolone. Multiple lesions in the brain responded to these modalities with complete resolution of the lesions and clinical improvement. In this case, impressive multiple and cystic lesions seen on brain MRI disappeared by whole brain irradiation and steroid. Since etiologies of multiple lesions on MRI includes many possibilities, histological diagnostic methods should be performed to obtain a definitive diagnosis. This is the first case of lymphomatoid granulomatosis that showed multiple and cystic lesions on brain MRI in Japan.
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PMID:[Lymphomatoid granulomatosis of the brain with multiple lesions on MRI]. 893 97


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