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

The term Morvan's disease, first coined in 1890, is still in use, although the generic term neuromyotonia--which is not exempt from criticism--has largely superseded it. Symptoms and signs are variable, ranging from benign painful fasciculations, pseudomyotonic cases, rigid forms, cases in which central nervous system features are also present (with, in addition to nerve hyperexcitability, agitation, confusion, delirium, insomnia, hyperhidrosis and tachycardia). A distal peripheral motor nerve is the origin of nerve hyperexcitability. There is growing evidence that autoimmunity is involved in the pathogenesis of many cases. Antibodies to voltage-gated potassium channels are detected in the serum of many patients with peripheral nerve hyperexcitability. Other cases are probably genetic. Inherited disorders are related to episodic dominant ataxia type 1, with the same mutation of a gene coding for potassium channel subunit Kv 1-1. Many inappropriate or non specific names are used to refer to peripheral nerve hyperexcitability. Isaacs syndrome, voltage-gated potassium channelopathy, or Morvan's syndrome are suggested.
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PMID:[From Morvan's disease to potassium channelopathies]. 1550 15

We report a case of serotonin syndrome that occurred in a patient with chronic heart failure associated with a panic disorder. The 39-year-old Japanese man had been treated with paroxetine at 20 mg/d for 1 1/2 years. He presented with rhabdomyolysis, renal failure, fulminant liver failure, cardiac conduction disturbance, and disseminated intravascular coagulation, as well as conventional symptoms of serotonin syndrome including alterations in cognition (disorientation, confusion) and behavior (restlessness), autonomic nervous system dysfunction (fever, shivering), and abnormal neuromuscular activity (ataxia, hyperreflexia, myoclonus). All medications prescribed before hospital admission were discontinued. After 24 hours of continuous venovenous hemofiltration, diuresis resumed and renal and liver function improved rapidly. Disorientation, restlessness, hyperreflexia, and myoclonus abated slowly over the next 72 hours. The patient's anxiety subsided more slowly, and he recovered completely 1 week later. The plasma concentration of paroxetine was elevated far above the upper limit of the therapeutic range. The patient had cytochrome P-450 (CYP) 2D6*1/*5, a heterozygosity of an inactivated allele of CYP2D6, which metabolizes paroxetine. The patient was determined to be an intermediate metabolizer who was potentially vulnerable to paroxetine, a major inhibitor of CYP2D6. Heart failure is often accompanied by psychiatric disorders. A wide range of drugs commonly prescribed for these conditions, including beta-blockers, antiarrhythmics, and antidepressants, are metabolized by CYP2D6. Genetic screening for CYP2D6 in patients with these conditions may prevent life-threatening drug intoxication.
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PMID:Life-threatening serotonin syndrome in a patient with chronic heart failure and CYP2D6*1/*5. 1554 25

The efficacies and ease of administration of four oral preanesthetic sedation protocols were compared in 18 adult, male rhesus macaques (Macaca mulatta) to achieve heavy sedation and alleviate anxiety, agitation, and potential trauma associated with remote anesthesia induction. The macaques, with average age and weight of 10 yr and 12.5 kg, respectively, were randomly assigned to one of four groups. Group 1 was given 10 mg/kg tiletaminezolazepam and 0.05 mg/kg medetomidine p.o., group 2 was given 1 mg/kg midazolam and 20 mg/kg ketamine p.o., group 3 was given 20 mg/kg ketamine and 0.05 mg/kg medetomidine p.o., and group 4 was given 3 mg/kg midazolam p.o. All protocols produced effects ranging from mild sedation to no response to noxious stimuli, depending on the success of administration. The mean interval to peak effect was 27-43 min in all groups. Ketamine and medetomidine provided significantly better sedation than midazolam alone; there were no other statistically significant differences among the four protocols. Oral tiletamine-zolazepam and medetomidine provided smooth, mild to moderate sedation with few side effects. The midazolam and ketamine combination resulted in severe ataxia. Orally administered ketamine and medetomidine provided smooth, easily reversible, heavy sedation leading to no response to noxious stimuli. Midazolam alone provided only mild sedation. No statistically significant differences in palatability of the four protocols were identified. Orally administered ketamine and medetomidine (group 3) provided the most consistently heavy sedation. A compounding pharmacy may be able to increase the palatability and level of acceptance of these combinations. Alternatively, oral midazolam syrup is well accepted by some animals and provides a mild sedative and calming effect, which may decrease stress associated with the induction of anesthesia via darting, pole syringes, etc.
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PMID:Four preanesthetic oral sedation protocols for rhesus macaques (Macaca mulatta). 1573 90

Video-polysomnographic monitoring of a female patient with spinocerebellar ataxia 3 (SCA-3) in whom rapid eye movement (REM) sleep behavior disorder was clinically suspected demonstrated recurrent episodes of unusual nocturnal motor restlessness occurring during non-REM sleep and severely disturbing both the patient and her bed partner's sleep. Even if nocturnal manifestations are common features of SCA-3 and increasingly recognized, such a case has never been reported before. We discuss this peculiar movement disorder as a particular manifestation of the wide spectrum of parasomnias occurring in such a rare neurodegenerative disease.
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PMID:Unusual nocturnal motor restlessness in a patient with spinocerebellar ataxia 3. 1579 16

Acute agitation occurs in a variety of medical and psychiatric conditions, and when severe can result in behavioural dyscontrol. Rapid tranquillisation is the assertive use of medication to calm severely agitated patients quickly, decrease dangerous behaviour and allow treatment of the underlying condition. Intramuscular injections of typical antipsychotics and benzodiazepines, given alone or in combination, have been the treatment of choice over the past few decades. Haloperidol and lorazepam are the most widely used agents for acute agitation, are effective in a wide diagnostic arena and can be used in medically compromised patients. Haloperidol can cause significant extrapyramidal symptoms, and has rarely been associated with cardiac arrhythmia and sudden death. Lorazepam can cause ataxia, sedation and has additive effects with other CNS depressant drugs.Recently, two fast-acting preparations of atypical antipsychotics, intramuscular ziprasidone and intramuscular olanzapine, have been developed for treatment of acute agitation. Intramuscular ziprasidone has shown significant calming effects emerging 30 minutes after administration for acutely agitated patients with schizophrenia and other nonspecific psychotic conditions. Intramuscular ziprasidone is well tolerated and has gained widespread use in psychiatric emergency services since its introduction in 2002. In comparison with other atypical antipsychotics, ziprasidone has a relatively greater propensity to increase the corrected QT (QTc) interval and, therefore, should not be used in patients with known QTc interval-associated conditions. Intramuscular olanzapine has shown faster onset of action, greater efficacy and fewer adverse effects than haloperidol or lorazepam in the treatment of acute agitation associated with schizophrenia, schizoaffective disorder, bipolar mania and dementia. Intramuscular olanzapine has been shown to have distinct calming versus nonspecific sedative effects. The recent reports of adverse events (including eight fatalities) associated with intramuscular olanzapine underscores the need to follow strict prescribing guidelines and avoid simultaneous use with other CNS depressants. Both intramuscular ziprasidone and intramuscular olanzapine have shown ease of transition to same-agent oral therapy once the episode of acute agitation has diminished. No randomised, controlled studies have examined either agent in patients with severe agitation, drug-induced states or significant medical comorbidity. Current clinical experience and one naturalistic study with intramuscular ziprasidone suggest that it is efficacious and can be safely used in such populations. These intramuscular atypical antipsychotics may represent a historical advance in the treatment of acute agitation.
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PMID:Pharmacological management of acute agitation. 1591 48

Most movement disorders, reflecting degenerative disorders, develop in a slowly progressive fashion. Some movement disorders, however, manifest with an acute onset. We wish to give an overview of the management and therapy of those acute-onset movement disorders.Drug-induced movement disorders are mainly caused by dopamine-receptor blockers (DRB) as used as antipsychotics (neuroleptics) and antiemetics. Acute dystonic reactions usually occur within the first four days of treatment. Typically, cranial pharyngeal and cervical muscles are affected. Anticholinergics produce a prompt relief. Akathisia is characterized by an often exceedingly bothersome feeling of restlessness and the inability to remain still. It is a common side effect of DRB and occurs within few days after their initiation. It subsides when DRB are ceased. Neuroleptic Malignant Syndrome is a rare, but life-threatening adverse reaction to DRB which may occur at any time during DRB application. It is characterised by hyperthermia, rigidity, reduced consciousness and autonomic failure. Therapeutically immediate DRB withdrawal is crucial. Additional dantrolene or bromocriptine application together with symptomatic treatment may be necessary. Paroxysmal dyskinesias are childhood onset disorders characterised by dystonic postures, chorea, athetosis and ballism occurring at irregular intervals. In Paroxysmal Kinesigenic Dyskinesia they are triggered by rapid movements, startle reactions or hyperventilation. They last up to 5 minutes, occur up to 100 times per day and are highly sensitive to anticonvulsants. In Paroxysmal Non-Kinesiogenic Dyskinesia they cannot be triggered, occur less frequently and last longer. Other paroxysmal dyskinesias include hypnogenic paroxysmal dyskinesias, paroxysmal exertional dyskinesia, infantile paroxysmal dystonias, Sandifer's syndrome and symptomatic paroxysmal dyskinesias. In Hereditary Episodic Ataxia Type 1 attacks of ataxia last for up to two minutes, may be accompanied by dysarthria and dystonia and usually respond to phenytoin. In Type 2 they can last for several hours, may be accompanied by vertigo, headache and malaise and usually respond to acetazolamide. Symptomatic episodic ataxias can occur in a number of metabolic disorders, but also in multiple sclerosis and Behcet's disease.
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PMID:Diagnosis and management of acute movement disorders. 1620 29

The clinicians usually do not consider syphilis in the differential diagnosis for patients with acute and chronic psychiatric symptoms. To familiarize clinicians particularly with neurosyphilis (NS) and to discuss the atypical antipsychotic alternatives, we wish to present a case with agitated, resistant psychotic symptoms related to neurosyphilis. The case was a 55-year-old male who has had anxiety, irritability, auditory hallucinations, ataxia, dysarthric speech, paranoid and persecutory delusions and agitated behaviour. Parenteral ziprasidone 20 mg/bid was initialized at the first day of admission to reduce agitation. Then it was switched to olanzapine velotab 10 mg/bid because of inefficacy. Parenteral cephtriaxon 1 g/daily was administered because of seropositive VDRl and TPHA and positive cerebrospinal fluid VDRl. Olanzapine velotab may be a good alternative antipsychotic and should be considered in reducing agitation and psychotic symptoms in NS.
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PMID:Effectiveness of olanzapine in neurosyphilis related organic psychosis: a case report. 1709 77

Few controlled studies are available to guide the clinician in treating potentially assaultive elderly individuals with psychiatric disorders. Safety concerns limit the use of benzodiazepines and antipsychotic medications in the elderly individual, making anticonvulsants an attractive alternative. This paper reviews three specific anticonvulsants for this purpose: gabapentin, oxcarbazepine and topiramate, describing safety and efficacy in elderly patients with severe agitation from psychosis or dementia. Gabapentin, renally excreted, with a half-life of 6.5-10.5 h, may cause ataxia. Oxcarbazapine, hepatically reduced, may cause hyponatremia, and topiramate may cause significant cognitive impairment. Nonetheless, these are important medications to consider in the treatment of agitation.
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PMID:Safety and efficacy of anticonvulsants in elderly patients with psychiatric disorders: oxcarbazepine, topiramate and gabapentin. 1736 59

In this report, we describe the case of two patients with Bickerstaff's brainstem encephalitis (BBE) who developed delirium manifested as emotional incontinence, restlessness, and aggressive behavior from disease onset. Serum anti-GQ1b and anti-GT1a IgG antibodies were detected in both patients. When unusual psychiatric symptoms are observed, in addition to acute ophthalmoplegia and ataxia, neurologists should take into account the possibility of BBE. Brain MRI findings were normal in both patients and SPECT was performed on only patient 1. SPECT of patient 1 showed reversible hypoperfusion in the brainstem, bilateral thalami, and medial frontal lobe. Brain SPECT appears to be useful for detecting lesions of the brainstem as well as the basal ganglia or cerebrum in BBE.
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PMID:Delirium in two patients with Bickerstaff's brainstem encephalitis. 1834 57

Beta-carboline alkaloids harmine, harmaline, and tetrahydroharmine can stimulate the central nervous system by inhibiting the metabolism of amine neurotransmitters, or by direct interaction with specific receptors; they are found in numerous plants, including Peganum harmala, Passiflora incarnata and Banisteriopsis caapi, and in the entheogen preparation Ayahuasca, which is traditionally brewed using B. caapi to enhance the activity of amine hallucinogenic drugs. The ingestion of plant preparations containing beta-carboline alkaloids may result in toxic effects, namely visual and auditory hallucinations, locomotor ataxia, nausea, vomiting, confusion and agitation. We report a case of intoxication following intentional ingestion of P. harmala seed infusion; P. harmala seeds were bought over the Internet. The harmala alkaloids were identified by gas chromatography-mass spectrometry in the seed extract and the patient's urine. This is, to our knowledge, the first case of P. harmala intoxication corroborated by toxicological findings.
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PMID:A case of beta-carboline alkaloid intoxication following ingestion of Peganum harmala seed extract. 1860 89


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