1/85. Huntington's disease and alcohol abuse.The dopamine, glutamate and GABA systems are known to mediate the effects of alcohol on the movement disorders, though their exact roles are not clear. Thus, use of alcohol has implications for pathogenesis as well as management of the movement disorders. These implications are discussed citing a patient who had a strong family history of Huntington's disease and in whom movement disorder and behavioral problems were manifest under alcohol use and withdrawal, but not while being abstinent.- - - - - - - - - - ranking = 1keywords = movement disorder, movement (Clic here for more details about this article) |
2/85. neuroacanthocytosis masquerading as Huntington's disease: CT/MRI findings.neuroacanthocytosis (NA) is a rare, degenerative, presumably autosomal-recessive disorder of the nervous system presenting in adulthood and is associated with acanthocytosis of the peripheral blood. The clinical spectrum of NA shares similarities with Huntington's disease (HD), including dyskinetic choreiform movements and degeneration of the caudate nucleus. A woman presented with choreiform movements and was given a presumed diagnosis of HD. neuroimaging studies were consistent with HD. She lacked the genetic marker for HD, and further evaluation revealed acanthocytosis of the peripheral blood. The case illustrates the similarities and differences in the clinical presentations and neuroimaging studies of these two disease entities, emphasizing the need for a careful clinical evaluation.- - - - - - - - - - ranking = 0.052112205351694keywords = movement (Clic here for more details about this article) |
3/85. Huntington's disease: review and anesthetic case management.Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depression, and apathy leads to cachexia. Factors of considerable concern to the anesthesiologist who treats patients with Huntington's disease may include how to treat frail elderly people incapable of cooperation, how to treat patients suffering from malnourishment, and how to treat patients with an increased risk for aspiration or exaggerated responses to sodium thiopental and succinylcholine. The successful anesthetic management of a 65-yr-old woman with Huntington's disease who presented for full-mouth extractions is described.- - - - - - - - - - ranking = 0.052112205351694keywords = movement (Clic here for more details about this article) |
4/85. Anaesthesia and juvenile Huntington's disease.Juvenile Huntington's disease (JHD) is an involuntary movement disorder that comprises both neurological and psychiatric symptoms. Whilst it has many similarities to Huntington's disease, it is regarded as a separate clinical entity. The anaesthetic plan should be based on careful assessment of the important issues, including the risk of regurgitation and pulmonary aspiration, possible associated autonomic neuropathy, poor respiratory function and the avoidance of precipitating convulsions and clonic spasms. We describe the management of a 12-year-old girl with JHD scheduled for gastroscopy under general anaesthesia necessitating the use of suxamethonium. We suggest an alternative mechanism for the delayed recovery seen in our patient and in other adult case reports.- - - - - - - - - - ranking = 0.33333333333333keywords = movement disorder, movement (Clic here for more details about this article) |
5/85. Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome.Huntington's disease is characterised by hyperkinetic movements, mainly chorea, cognitive dysfunction, and psychiatric abnormalities. Non-dopa responsive parkinsonism occurs in the later stages of choreic disease or as the predominant feature of juvenile patients (Westphal variant). Late onset Huntington's disease presenting as levodopa responsive parkinsonism is rare. A series of four patients with late onset Huntington's disease presenting as levodopa responsive parkinsonism and cardiovascular dysautonomia, initially misdiagnosed as multiple system atrophy (MSA) in three patients, is reported. levodopa treatment did not unmask significant chorea. These cases suggest the presence of a distinct phenotypic variant of Huntington's disease to be added to the differential diagnosis of other akinetic rigid syndromes.- - - - - - - - - - ranking = 0.026056102675847keywords = movement (Clic here for more details about this article) |
6/85. An autopsy case with clinically and molecular genetically diagnosed Huntington's disease with only minimal non-specific neuropathological findings.An autopsy case with clinically and molecular genetically diagnosed Huntington's disease (HD) accompanied with minimal non-specific neuropathological features was reported. When the patient was 45 years old, he had faulty memory, mood swing, personality change and agitation. Neurological and psychiatric examinations revealed choreoathetoid movements in limbs and trunk, generalized hyperreflexia and mental deterioration. However, cerebellar ataxia and muscle rigidity were not disclosed. neuroimaging study did not show a definite atrophy of heads of caudate nuclei. neuroacanthocytosis and Wilson's disease were ruled out by the peripheral blood examination and serum Cu and ceruloplasmin examination. At the age of 55 he died of pneumonia. Post-mortem examination revealed minimal non-specific neuropathological features for HD (Vonsattel's grade 0), that is, no visible fibrillary gliosis in the striatum, and few neuronal loss and only proliferation of astrocytes (astrocytosis) in the striatum. Molecular-genetic study the patient's brain tissues and his youngest son's blood was performed. These studies revealed 40 CAG repeats in the patient, 56 CAG repeats in his youngest son. These results suggest they may be HD. Vonsattel et al. [ 1998] insist that grade 0 comprises 1% of all HD brains, and grade 1 comprises 4% of all HD brains. But we could not find any reports in which the clinical and neuropathological features were described in detail on the cases with clinically and molecular genetically diagnosed HD without specific pathological findings. Therefore, we present in detail the clinical and neuropathological features of such case.- - - - - - - - - - ranking = 0.026056102675847keywords = movement (Clic here for more details about this article) |
7/85. brain SPECT imaging in Huntington's disease before and after therapy with olanzapine. Case report.Olanzapine, an atypical antipsychotic drug, was administered to a patient with Huntington's disease (HD) with marked choreiform movements. brain SPECT with 99mTc-HMPAO was performed before and after treatment. brain SPECT imaging has been performed in patients with HD in order to determine the status of basal ganglia perfusion. The use of brain SPECT with 99mTc-HMPAO before and after treatment in patients with HD has not been yet reported. The marked hypoperfusion of the basal ganglia on brain SPECT performed before therapy with olanzapine improved significantly after treatment.- - - - - - - - - - ranking = 0.026271726362989keywords = movement, status (Clic here for more details about this article) |
8/85. Tourettism as clinical presentation of Huntington's disease with onset in childhood.Infantile Huntington's disease (HD) shows a wide clinical heterogeneity. Here we describe the case of a child affected by HD who showed unusual neurological features consistent with tourettism. The absence of family history and persisting normal magnetic resonance imaging (MRI) results long after the onset of symptoms delayed the diagnosis of the disease. An MRI exam performed 26 months after disease onset disclosed bilateral atrophy in the putamen, suggesting HD. The diagnosis was confirmed by genetic analysis. The present report underlines the need to consider HD in childhood cases of unusual and even unfamiliar progressive movement disorders.- - - - - - - - - - ranking = 0.33333333333333keywords = movement disorder, movement (Clic here for more details about this article) |
9/85. Psychiatric presentation of Huntington's disease in a Malaysian family.A 32-year-old Chinese lady presented to the Psychiatric Clinic with a history of change in personality for 2 years and abnormal movements for a year. After thorough investigations and observation a diagnosis of Huntington's disease was made. Her elder brother was traced and found to have Huntington's disease as well. He had a long standing history of antisocial behaviour and substance abuse long before the onset of the choreiform movements. Her younger brother also has choreiform movements for the last 2 years and had recent change in personality. Their mother also had abnormal movements and was recorded to be depressed and attempted suicide. The maternal grandfather had a mental illness and was warded at a mental institution till his death in 1942. Psychiatric presentation of Huntington's disease in this Malaysian family is prominent and preceded the characteristic movements in the present generation.- - - - - - - - - - ranking = 0.13028051337923keywords = movement (Clic here for more details about this article) |
10/85. De novo expansion of a CAG repeat in a Japanese patient with sporadic Huntington's disease.A 49-year-old man was admitted to our hospital complaining of dysarthria and involuntary movements of his neck and extremities. He had first begun to experience involuntary neck movements at the age of 40 and his symptoms gradually progressed thereafter. There was no family history of neurological disorders. On admission he showed memory disturbance, dysarthria, and choreic movements. The involuntary movements affected his face, neck, trunk, and extremities. MRI of the brain revealed atrophy of both the cerebral cortex and the head of the caudate nucleus. dna samples for molecular analysis were obtained from the patient and both of his parents. In this pedigree, the father carried a premutated allele of 35 CAG repeats and transmitted an expanded allele of 43 CAG repeats to his son. paternity and maternity were analyzed using a microsatellite marker located in a different chromosome. To our knowledge, this is the first report of a sporadic case of Huntington's disease in a non-caucasian population in which the disease prevalence is much lower than that in the caucasian population. A new mutation in the current Japanese population which shares the same mechanism as de novo mutation in Caucasians may have contributed to the frequency of HD in japan at the present time.- - - - - - - - - - ranking = 0.10422441070339keywords = movement (Clic here for more details about this article) |
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