Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The type I Chiari malformation consists of a caudal displacement of the cerebellar tonsils through the foramen magnum into the cervical spinal canal. The most common presenting symptoms, such as pain, weakness and headache, are frequently preceded by otoneurological symptoms. Sensorineural hearing loss, vertigo, nystagmus, dysequilibrium, tinnitus and other cranial nerve involvement have been reported in Chiari-I malformation. A case report is presented and the clinical features of the disease are discussed with emphasis on the otoneurological aspects.
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PMID:Otoneurological manifestations in Chiari-I malformation. 832 27

We report a case of basilar artery migraine with transient MRI and EEG abnormalities. A 25-year-old male medical student developed a right occipital throbbing headache one hour after the abrupt onset of vertigo, nausea, left-sided homonymous hemianopsia and nystagmus. All of his complaints subsided the following day. He had suffered from attacks of headache accompanied by the same type of aural symptoms almost every ten days. He was therefore diagnosed as having basilar artery migraine. CT scans during the period of recurrent migraine demonstrated no abnormalities, while T2-weighted MR images revealed a high signal intensity region in the right occipital cortex and adjacent white matter perfused by rami temporales of the right posterior cerebral artery. Cerebral hypoperfusion was detected by a SPECT scanner in the right posterior region, where EEG recorded during the migraine attack demonstrated paroxysmal-localized sharp waves. MRI and EEG performed two months after the last attack showed no obvious abnormalities along with clinical disappearance of the migraine attacks. We found few reports of transient MRI changes associated with migraine. This case suggests that transient changes in MR images reflect vasogenic edema caused by migraine.
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PMID:[Basilar artery migraine with transient MRI and EEG abnormalities]. 833 77

Psychogenic dizziness is defined as recurring or persistent symptoms of balance dysfunction, inconsistent with organic vestibular disease as determined by history, clinical examination and pertinent investigations, and consistent with emotional origin. Of 1,335 patients seen in our dizziness clinic between January 1988 and August 1991, psychogenic dizziness was diagnosed in 180 (13.5%) patients. There were 67 men and 113 women aged from 12 to 77 years (mean age 40.2 years). The characteristics of psychogenic dizziness are: (1) continuous dizziness for long periods of time; (2) younger patients; (3) predominant female; (4) associated symptoms of panic attack, such as headache, breathlessness, nausea, sleep disturbance, paresthesias, anxiety and palpitation; (5) symptoms of aggravation due to stressful life events; (6) normal neurotological bedside examination; (7) hyperventilation reproduced accurately. The electronystagmographic results of 74 patients show normal bithermal caloric responses in 47 patients (63.5%), caloric hyperactivity in 21 patients (28.4%), canal paresis in four patients (5.4%), canal paresis with directional preponderance in two patients (2.7%), large random voluntary eye swings or severe blinking in 35 patients (47.3%), and spontaneous nystagmus (slow phase velocity < 6.5 degrees/s) in four patients (5.4%). There were 31 patients who consulted psychiatrists with diagnoses of anxiety (51.6%), depression (16.1%), insomnia (12.9%), psychosomatic disorder and adjustment disorder. Treatment of patients with psychogenic dizziness must be directed at the underlying anxiety. Psychiatric consultation is necessary.
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PMID:[Psychogenic dizziness]. 848 48

A case of cerebellar pilocytic astrocytoma is reported. This tumor occurs typically in the first two decades of life and is seldom reported in adults. The 42-year-old patient presented with occipital headaches, nausea, and unsteady gait. Nystagmus and right dysmetria were noted. A CT scan showed a hypodense, nonenhancing, voluminous, right hemispheric cerebellar cyst. Magnetic resonance imaging showed a nodule in the wall of the cyst which became hyperintense with gadolinium. The mass was resected through a small occipital craniotomy. Neuropathological examination revealed a juvenile pilocytic astrocytoma.
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PMID:Pilocytic cerebellar astrocytoma in adults: case report. 849 94

Degenerative changes of the cervical spine often cause the pains and vertigo as well as pathological results of otoneurological tests. The aim of the study was to estimate the frequency of the symptoms occurrence in the group of 146 patients, 63 male and 83 female, with degenerative changes of the cervical spine. Permanent or periodical headaches of various intensity occurred in 60% of the patients. Balance disturbances was observed in 74% of the patients, specially in women. In the cervical spine X-ray there were observed most frequently marginal degenerations with osteophytosis (16%), intervertebral disk narrowing (10%), and shallowing of the physiological lordosis (11%). In ENG examination, positional nystagmus (42-38%), gaze nystagmus (14-23%) were mainly recorded, with the eyes opened and closed. The eye-tracking pattern test was pathological in 57%. Optokinetic test results showed pathological records in 32% of the patients, unilateral weakness was observed in 22% and bilateral weakness in 17% of the patients. Observations mentioned above confirm frequent pathology and the necessity of early diagnosis and treatment of patients with degenerative changes of the cervical spine.
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PMID:[The examination of vestibular system in patients with degenerative changes of the cervical spine]. 853 53

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 rare case of posterior fossa dural arteriovenous fistula with cerebellar circulation disorder is reported. A 64-year-old male was admitted to a hospital with complaints of nausea, vertigo and mild headache. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed cerebellar infarction. Then, he was sent to our hospital. He had complaints of cerebellar signs and nystagmus. Cerebral angiography showed dural AVF of the posterior fossa and occlusion of the left transverse-sigmoid sinuses. Because his condition deteriorated, embolization of the left middle meningeal artery, ascending pharyngeal artery and occipital artery was performed selectively on August 18. Subsequently, isolation of dural AVF was performed on August 19. After the operation he improved and was able to walk with a stick. Controlled angiography revealed the disappearance of dural AVF. This case suggested that AV shunt is essential for the progression of venous infarction.
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PMID:[Posterior fossa dural arteriovenous fistula with progressive disorder of cerebellar circulation; case report]. 869 72

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

The clinical neurological and electroneuromyographycal examination were performed in 75 patients with chronic alcoholism including 15 patients with abstinence (withdrawal) syndrome. The abstinence period without any alcohol consumption did not last more that 6 days before observation time. The clear, specific neurological symptoms were revealed in alcohol abstinence syndrome (AAS), exactly: the general brain disturbances in the form of headache, dizziness, horizontal small-swinging nystagmus, dynamic ataxia, the increase of tendinous reflexes preferentially from upper limbs, the tremor of head, tongue and of streched out arms fingers, the sympathic adrenal type vegetative disturbances. The increase of impulse conduction rate along the median nerve as well as elevation of craniocaudal coefficient and neuromuscular conduction disturbances were also characteristic for AAS.
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PMID:[Neurological and neurophysiological aspects of the alcohol abstinence syndrome]. 878 80

Amyloid deposits in leptomeningeal vessels, subarachnoid, subpial, and subependymal cerebrospinal regions, spinal ganglia, peripheral nerves, and some internal organs (predominantly heart and kidney) characterize a dominantly inherited disease in a Hungarian family. We found four definitely and three probably affected members in this family of 56 persons in four generations. Clinical features in all definitely diseased patients include disturbance of memory, psychomotor deceleration, ataxia, and hearing loss. In most patients there was temporary disorientation, migraine-like headache with vomiting, and tremor. Some patients had nystagmus, pyramidal signs with spastic paraparesis, hallucinations, urinary retention, and obstipation. Single patients had facial tics and sleep disorders. Progressive visual disturbance and clinically manifest polyneuropathy were absent. CSF protein was markedly elevated in all patients. CT showed characteristic symmetric calcification along the sylvian fissure; MRI after contrast administration showed prominent enhancement at the surface of the sylvian fissures, brainstem, and cerebellum. Autopsy data was available in three definitely affected patients and in one unaffected family member. Immunohistochemistry identified the amyloid deposits as of the AF (transthyretin, TTR) type; DNA studies revealed a novel TTR missense mutation at codon 18 (TTR Asp18Gly). According to clinical features, pathologic alterations, and molecular studies, this disease is a novel type of systemic familial amyloidosis with disease manifestation clinically restricted to the CNS. It is similar to the oculoleptomeningeal amyloidoses but can be clinically diagnosed by characteristic CTs and the absence of progressive visual impairment.
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PMID:Familial meningocerebrovascular amyloidosis, Hungarian type, with mutant transthyretin (TTR Asp18Gly) 896 Jul 46


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