Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An open-label, dose-escalation study was conducted to determine doses of lorazepam required to induce anterograde amnesia and sedation in children without producing excessive toxicity. Oncology patients 4 to 17 years of age undergoing lumbar puncture or bone marrow aspiration were eligible; a patient could be entered in the study for a second procedure at a different lorazepam dose. A single oral dose of lorazepam was administered 45-60 minutes before the procedure. Starting with 0.02 mg/kg, the same dose was given to three patients; if no dose-limiting effects occurred, dose was increased by 0.01 mg/kg. Before the procedure the patient was shown a toy that he or she was later asked to identify. Immediately after the procedure (usually 60-75 minutes after the lorazepam dose), sedation was assessed on a scale of 0 (alert) to 4 (coma), and the clinician performing the procedure was asked to subjectively evaluate sedation. Patients were rated for amnesia 24 hours after the procedure; a scale of 0 (recalls procedure and toy without prompting) to 4 (recalls nothing since procedure) was used. Twenty patients received 28 doses of lorazepam. The study was terminated when two patients who received 0.10 mg/kg had excessive ataxia. Sedation was subjectively considered adequate for 24 of the procedures. Sedation and amnesia scores were not well correlated with increased dose. Amnesia occurred in some patients with doses as low as 0.03 mg/kg. In children undergoing lumbar puncture or bone marrow aspiration, premedication with oral lorazepam 0.02-0.09 mg/kg generally produced adequate sedation for the procedure.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Determination of sedative and amnestic doses of lorazepam in children. 193 19

300 women aged 16-37 received analgesic-sedative preparations for miniabortions preprandially after admittance to the clinic. Anamnesis was followed by routine blood pressure check, then 0.01 mg/kg-1 atropine, 0.1 mg/kg-1 diazepam iv, or 0.05 mg/kg-1 midazolam iv was given to 25 women. 1-2 minutes later 0.5 mg/kg-1 ketamine iv was given, and at the time of insertion of the aspiration cannula into the cervix, another dose of 0.25 mg/kg-1 of ketamine was given iv. The total dose did not exceed 1 mg/kg-1 iv. All patients also received a ketamine-benzodiazepine combination of analgesia prior to the miniabortion procedure. In psychic, unstable women anxiolytic effects were apparent, but in all of them vertigo and the sensation of floating ensued with horizontal and vertical nystagmus, although information could be extracted from them depending on the degree of analgesia. Anterograde amnesia followed, and the systolic and diastolic pressure increased. The maximum duration of analgesic effect was 3-5 minutes, most women became well-oriented without ataxia and returned home 4 hours after the operation. No psychic effects lasted, and nausea or vomiting was minimal. The hallucinogenic effect of ketamine was attributable to the stimulation of the central dopaminergic system, while diazepam (Spofa) influenced the limbic system causing anterograde amnesia. The potential of midazolam-benzodiazepine combination for future sue lies in its very short biological half-life (1.5-2.5 hours) compared with diazepam (24-36 hours).
 ...
PMID:[Analgesia-sedation using ketamine and benzodiazepines in mini-abortion]. 271 9

Forty female out-patients undergoing therapeutic abortion participated in a double-blind study comparing flunitrazepam 0.05 mg . kg-1 with thiopentone 6.0 mg . kg-1 as induction agents for general anaesthesia. Induction time, as measured by the time to loss of lid reflex and voluntary speech, was not only significantly longer in patients receiving flunitrazepam, but also much more variable and imprecise than with thiopentone. The Steward recovery room scores and psychomotor drawing test results revealed that recovery was significantly slower in the flunitrazepam group. Anterograde amnesia was observed in all patients who had received flunitrazepam and in one patient who had received thiopentone. No retrograde amnesia was found in either group. Flunitrazepam produced postoperative drowsiness, sedation, ataxia and nausea while with thiopentone discomfort from surgery and discomfort at the intravenous injection site were the main complaints. Because of the slowness of induction with flunitrazepam and marked individual variation, we do not feel that this drug can be considered a suitable agent for routine induction of general anaesthesia.
 ...
PMID:Comparison of flunitrazepam and thiopentone for induction of general anaesthesia. 610 7

The case reported here is that of a woman, without any significant pathological antecedent. At age 54, she developed signs of thalamic dementia and died 5 years later. The prominent symptoms were massive anterograde amnesia, apathia, apragmatism, ataxia and a frontal syndrome. She never showed aphasia, apraxia, agnosia or disorders of ocular motility. The C. T. Scan showed lacunar low densities in the mesencephalon and both thalami, mainly on the left side, as well as hydrocephalus caused by a stenosis of the aqueduct as shown by other neuroradiological procedures. The neuropathological examination showed space-occupying lacunae, bulging in the third ventricle, squeezing the aqueduct and protruding into the fourth ventricle. These lacunae were situated in the territory of the paramedian mesencephalo-thalamic arterial pedicle. They were perivascular spaces distensions, probably caused by disorder of the permeability of the arterial wall. This hypothesis is supported by the presence of severe lesions of segmental necrotizing angeitis on a paramedian mesencephalic artery. The etiology of this angeitis is unknown. As far as we know, such neuropathological lesions have never been reported previously. Therefore the pathology and etiopathogeny of cerebral lacunae should be reconsidered.
 ...
PMID:[Thalamic dementia. Expansive lacunae of the thalamo-paramedian mesencephalic area. Hydrocephalus caused by stenosis of the aqueduct of Sylvius]. 613 53

A 65-year-old woman with progressive visuospatial dysfunction for 2 years complained of later-onset associated memory impairment. MRI revealed diffuse cerebrocortical atrophy, which was especially severe in both parieto-occipital regions but spared the calcarine and pericalcarine cortices. Examination 5 years after onset revealed left visual hemi-neglect, oculomotor apraxia, optic ataxia, simultanagnosia, verbal alexia, lexical and spatial agraphia, and anterograde amnesia. This patient's disorder is considered in the context of previous reports on the array of cognitive disturbances associated with posterior cortical atrophy (pCA). Special emphasis is made on her reading and writing disturbances, because their prevalence and range of individual variability have not been established in pCA. This array of neuropsychological manifestations may help to distinguish among different clinical and etiological types of pCA, and to elucidate the pathophysiology of a syndrome that has been associated with conditions as diverse as Alzheimer's disease, subcortical gliosis, and prion diseases. The parameters described in our case may thus help to address these issues in clinico-pathological studies with large numbers of patients with pCA.
 ...
PMID:Alexia and agraphia in posterior cortical atrophy. 911 97

A 26-year-old woman suffered disseminated intravascular coagulation (DIC) and a brief respiratory arrest following recreational use of 3,4-methylene-dioxymethamphetamine (MDMA; 'ecstasy'), together with amyl nitrate, lysergic acid (LSD), cannabis and alcohol. She was left with residual cognitive and physical deficits, particularly severe anterograde memory disorder, mental slowness, severe ataxia and dysarthria. Follow-up investigations have shown that these have persisted, although there has been some improvement in verbal recognition memory and in social functioning. Magnetic resonance imaging and quantified positron emission tomography investigations have revealed: (i) severe cerebellar atrophy and hypometabolism accounting for the ataxia and dysarthria; (ii) thalamic, retrosplenial and left medial temporal hypometabolism to which the anterograde amnesia can be attributed; and (iii) some degree of fronto-temporal-parietal hypometabolism, possibly accounting for the cognitive slowness. The putative relationship of these abnormalities to the direct and indirect effects of MDMA toxicity, hypoxia and ischaemia is considered.
 ...
PMID:Amnesic syndrome and severe ataxia following the recreational use of 3,4-methylene-dioxymethamphetamine (MDMA, 'ecstasy') and other substances. 1174 84

A 38-year-old male presented with a three-week history of bilateral lower extremity choreiform movements. History included sleep abnormalities, rushed and unintelligible speech, with delusions two to six months prior to presentation. He also developed mild dysphagia, staring spells, and anterograde amnesia. On examination, he had pressured speech, asynchronous cycling movements of the bilateral lower extremities persisting during sleep, occasional ballistic movements of the upper extremities, and ataxia. Magnetic resonance imaging (MRI) of the brain showed high cortical signal change in bilateral parieto-occipital cortices with evidence of medullary olive hypertrophy bilaterally. Electroencephalography showed generalized slowing without periodic spikes. Cerebrospinal fluid was positive for protein 14-3-3 and real-time quaking-induced conversion. Genetic testing was positive for autosomal dominant prion protein gene (PRNP) genetic mutation. The patient passed away three months after discharge. This case provides previously undescribed imaging and movement abnormalities in a patient with familial Creutzfeldt-Jakob disease (CJD), and suggests that CJD should not be removed from the differential in patients with these atypical findings.
 ...
PMID:Hypertrophic Olivary Degeneration and Movement Disorder in a Patient with Familial Creutzfeldt-Jakob Disease. 3317 8