Kluver-Bucy syndrome in Huntington's chorea. 1985

A Janati

In this paper the first case of Kluver-Bucy syndrome (KBS) in Huntington's chorea is reported. The patient, a 46-year-old man with advanced Huntington's disease, displayed prosopagnosia, oral tendencies, emotional changes, hypersexual behavior, and hyperphagia associated with severe dementia. Haloperidol in moderate doses controlled both the KBS and the chorea, suggesting a possible role for the dopaminergic system in the pathogenesis of KBS in Huntington's disease. The presence of profound dementia in our patient supports the previous assertion that human cases of KBS are invariably associated with severe cognitive dysfunction. Since KBS was established as an entity, a great deal of attention has been directed to its neuroanatomical basis. However, due to the multidetermined nature of human behavior, the role of physiological, psychological, and environmental factors should also be taken into consideration with regard to the pathogenesis of this syndrome.

UI MeSH Term Description Entries
D008297 Male Males
D009913 Oral Stage The earliest of the stages of infantile psychosexual development, lasting from birth to 12 months or longer. Oral Erotic Stage,Oral Phase,Oral Sadistic Stage,Oral Erotic Stages,Oral Sadistic Stages,Stage, Oral Erotic,Stage, Oral Sadistic
D003704 Dementia An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness. Senile Paranoid Dementia,Amentia,Familial Dementia,Amentias,Dementia, Familial,Dementias,Dementias, Familial,Dementias, Senile Paranoid,Familial Dementias,Paranoid Dementia, Senile,Paranoid Dementias, Senile,Senile Paranoid Dementias
D004298 Dopamine One of the catecholamine NEUROTRANSMITTERS in the brain. It is derived from TYROSINE and is the precursor to NOREPINEPHRINE and EPINEPHRINE. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of receptors (RECEPTORS, DOPAMINE) mediate its action. Hydroxytyramine,3,4-Dihydroxyphenethylamine,4-(2-Aminoethyl)-1,2-benzenediol,Dopamine Hydrochloride,Intropin,3,4 Dihydroxyphenethylamine,Hydrochloride, Dopamine
D005247 Feeding Behavior Behavioral responses or sequences associated with eating including modes of feeding, rhythmic patterns of eating, and time intervals. Dietary Habits,Eating Behavior,Faith-based Dietary Restrictions,Feeding Patterns,Feeding-Related Behavior,Food Habits,Diet Habits,Eating Habits,Behavior, Eating,Behavior, Feeding,Behavior, Feeding-Related,Behaviors, Eating,Behaviors, Feeding,Behaviors, Feeding-Related,Diet Habit,Dietary Habit,Dietary Restriction, Faith-based,Dietary Restrictions, Faith-based,Eating Behaviors,Eating Habit,Faith based Dietary Restrictions,Faith-based Dietary Restriction,Feeding Behaviors,Feeding Pattern,Feeding Related Behavior,Feeding-Related Behaviors,Food Habit,Habit, Diet,Habit, Dietary,Habit, Eating,Habit, Food,Habits, Diet,Pattern, Feeding,Patterns, Feeding,Restrictions, Faith-based Dietary
D006220 Haloperidol A phenyl-piperidinyl-butyrophenone that is used primarily to treat SCHIZOPHRENIA and other PSYCHOSES. It is also used in schizoaffective disorder, DELUSIONAL DISORDERS, ballism, and TOURETTE SYNDROME (a drug of choice) and occasionally as adjunctive therapy in INTELLECTUAL DISABILITY and the chorea of HUNTINGTON DISEASE. It is a potent antiemetic and is used in the treatment of intractable HICCUPS. (From AMA Drug Evaluations Annual, 1994, p279) Haldol
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006816 Huntington Disease A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4) Huntington Chorea,Juvenile Huntington Disease,Akinetic-Rigid Variant of Huntington Disease,Chorea, Chronic Progressive Hereditary (Huntington),Chronic Progressive Hereditary Chorea (Huntington),Huntington Chronic Progressive Hereditary Chorea,Huntington Disease, Akinetic-Rigid Variant,Huntington Disease, Juvenile,Huntington Disease, Juvenile-Onset,Huntington Disease, Late Onset,Huntington's Chorea,Huntington's Disease,Juvenile-Onset Huntington Disease,Late-Onset Huntington Disease,Progressive Chorea, Chronic Hereditary (Huntington),Progressive Chorea, Hereditary, Chronic (Huntington),Akinetic Rigid Variant of Huntington Disease,Chorea, Huntington,Chorea, Huntington's,Huntington Disease, Akinetic Rigid Variant,Huntington Disease, Juvenile Onset,Huntington Disease, Late-Onset,Juvenile Onset Huntington Disease,Late Onset Huntington Disease
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000377 Agnosia Loss of the ability to comprehend the meaning or recognize the importance of various forms of stimulation that cannot be attributed to impairment of a primary sensory modality. Tactile agnosia is characterized by an inability to perceive the shape and nature of an object by touch alone, despite unimpaired sensation to light touch, position, and other primary sensory modalities. Auditory Agnosia,Finger Agnosia,Sensory Agnosia,Tactile Agnosia,Visual Agnosia,Agnosia for Pain,Agnosia for Smell,Agnosia for Taste,Agnosia for Temperature,Anosognosia,Auditory Agnosia, Congenital,Body-Image Agnosia,Congenital Auditory Agnosia,Developmental Agnosia,Gustatory Agnosia,Ideational Agnosia,Olfactory Agnosia,Position Agnosia,Somatosensory Agnosia,Time Agnosia,Topographical Agnosia,Visual Agnosia for Objects,Visual Disorientation Syndrome,Visuospatial Agnosia,Agnosia for Tastes,Agnosia, Auditory,Agnosia, Body-Image,Agnosia, Congenital Auditory,Agnosia, Developmental,Agnosia, Finger,Agnosia, Gustatory,Agnosia, Ideational,Agnosia, Olfactory,Agnosia, Position,Agnosia, Sensory,Agnosia, Somatosensory,Agnosia, Tactile,Agnosia, Time,Agnosia, Topographical,Agnosia, Visual,Agnosia, Visuospatial,Agnosias,Agnosias, Auditory,Agnosias, Body-Image,Agnosias, Congenital Auditory,Agnosias, Developmental,Agnosias, Finger,Agnosias, Ideational,Agnosias, Olfactory,Agnosias, Position,Agnosias, Sensory,Agnosias, Somatosensory,Agnosias, Tactile,Agnosias, Time,Agnosias, Topographical,Agnosias, Visual,Agnosias, Visuospatial,Anosognosias,Auditory Agnosias,Auditory Agnosias, Congenital,Body Image Agnosia,Body-Image Agnosias,Congenital Auditory Agnosias,Developmental Agnosias,Finger Agnosias,Ideational Agnosias,Olfactory Agnosias,Position Agnosias,Sensory Agnosias,Somatosensory Agnosias,Syndrome, Visual Disorientation,Syndromes, Visual Disorientation,Tactile Agnosias,Time Agnosias,Topographical Agnosias,Visual Agnosias,Visual Disorientation Syndromes,Visuospatial Agnosias

Related Publications

A Janati
January 2005, The Journal of the Association of Physicians of India,
A Janati
May 2003, The American journal of emergency medicine,
A Janati
September 1997, Journal of neurosurgical sciences,
A Janati
January 1997, Acta neurochirurgica,
A Janati
January 1987, Pediatric neurology,
A Janati
January 2000, Indian journal of pediatrics,
A Janati
December 1998, Clinical neurology and neurosurgery,
A Janati
July 2004, Neurology,
A Janati
April 2001, CNS spectrums,
A Janati
February 1984, The Journal of clinical psychiatry,
Copied contents to your clipboard!