[Clinical heterogeneity of Alzheimer's disease. Different clinical profiles can predict the progression rate]. 2004

C A Mangone
Centro de Asistencia e Investigación del Deterioro Cognitivo, Hospital Santojani, Buenos Aires, Argentina. camangone@fibertel.com.ar

BACKGROUND Alzheimer's disease (AD) is a degenerative dementia that may disclose different cognitive, behavioral, psychiatric and functional symptoms since onset. These distinct cognitive profiles support the conception of clinical heterogeneity and account for AD's highly variable rate of progression. In spite of strict diagnostic criteria NINCS ADRDA's and DSM IV the clinical certainty is only about 85%. Mayeux define 4 subtypes: a). Benign: mild cognitive and functional impairment without focal signs and late onset behavioral signs, slow progression; b). Myoclonic: usually of presenile onset with severe cognitive deterioration, mutism and early onset myoclonus; c). Extrapyramidal: early onset akineto rigid signs with severe cognitive, behavioral and psychiatric involvement; d). Typical: gradual and progressive cognitive, behavioral and functional impairment. The differentiation of these subtypes will allow us to define discrete patterns of progression, to define prognostic subgroups, and to homogenize them for clinical research and drug trials. METHODS We examined 1000 charts of probable AD patients from the Santojanni Center. We found 42% extrapyramidal, 35% typical, 15% benign and 8% myoclonic. The early onset of parkinsonism and myoclonus predict a rapidly evolving cognitive impairment and a more severe rate of progression with psychiatric disorders and dependency in activities of daily living. (DADL) Patients with low level of education, low cognitive performance at entry as well as those with rapid rate of cognitive deterioration had a faster rate of progression to DADL. CONCLUSIONS Delusions, low level of education, extrapyramidal signs and motor hyperactivity but not hallucinations, and anosognosia were the best non cognitive predictors of DADL.

UI MeSH Term Description Entries
D009207 Myoclonus Involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles. This condition may be a feature of some CENTRAL NERVOUS SYSTEM DISEASES; (e.g., EPILEPSY, MYOCLONIC). Nocturnal myoclonus is the principal feature of the NOCTURNAL MYOCLONUS SYNDROME. (From Adams et al., Principles of Neurology, 6th ed, pp102-3). Myoclonus, Action,Myoclonus, Nocturnal,Myoclonus, Palatal,Polymyoclonus,Myoclonic Jerk,Myoclonic Jerking,Myoclonus Simplex,Myoclonus, Eyelid,Myoclonus, Intention,Myoclonus, Lower Extremity,Myoclonus, Oculopalatal,Myoclonus, Segmental,Myoclonus, Sleep,Myoclonus, Upper Extremity,Action Myoclonus,Extremity Myoclonus, Lower,Extremity Myoclonus, Upper,Eyelid Myoclonus,Intention Myoclonus,Jerk, Myoclonic,Jerking, Myoclonic,Jerks, Myoclonic,Lower Extremity Myoclonus,Myoclonic Jerks,Nocturnal Myoclonus,Oculopalatal Myoclonus,Palatal Myoclonus,Segmental Myoclonus,Simplex, Myoclonus,Sleep Myoclonus,Upper Extremity Myoclonus
D009460 Neurologic Examination Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system. Examination, Neurologic,Neurological Examination,Examination, Neurological,Examinations, Neurologic,Examinations, Neurological,Neurologic Examinations,Neurological Examinations
D010468 Perceptual Disorders Cognitive disorders characterized by an impaired ability to perceive the nature of objects or concepts through use of the sense organs. These include spatial neglect syndromes, where an individual does not attend to visual, auditory, or sensory stimuli presented from one side of the body. Hemispatial Neglect,Hemisensory Neglect,Sensory Neglect,Somatosensory Discrimination Disorder,Discrimination Disorder, Somatosensory,Discrimination Disorders, Somatosensory,Hemisensory Neglects,Hemispatial Neglects,Neglect, Hemisensory,Neglect, Hemispatial,Neglect, Sensory,Neglects, Hemisensory,Perceptual Disorder,Sensory Neglects,Somatosensory Discrimination Disorders
D002531 Cerebellum The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills. Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D004522 Educational Status Educational attainment or level of education of an individual. Education Level,Educational Achievement,Educational Attainment,Educational Level,Level of Education,Maternal Education Level,Maternal Educational Attainment,Maternal Educational Level,Paternal Education Level,Paternal Educational Attainment,Paternal Educational Level,Achievement, Educational,Educational Status, Maternal,Educational Status, Paternal,Maternal Educational Status,Paternal Educational Status,Status, Educational,Attainment, Educational,Attainment, Maternal Educational,Attainment, Paternal Educational,Education Level, Maternal,Education Level, Paternal,Education Levels,Educational Achievements,Educational Attainment, Maternal,Educational Attainment, Paternal,Educational Attainments,Educational Level, Maternal,Educational Level, Paternal,Educational Levels,Level, Education,Level, Educational,Level, Maternal Education,Level, Maternal Educational,Level, Paternal Education,Level, Paternal Educational,Levels, Maternal Educational,Maternal Education Levels,Maternal Educational Attainments,Maternal Educational Levels,Paternal Education Levels,Paternal Educational Attainments,Paternal Educational Levels,Status, Maternal Educational,Status, Paternal Educational
D005625 Frontal Lobe The part of the cerebral hemisphere anterior to the central sulcus, and anterior and superior to the lateral sulcus. Brodmann Area 8,Brodmann's Area 8,Frontal Cortex,Frontal Eye Fields,Lobus Frontalis,Supplementary Eye Field,Area 8, Brodmann,Area 8, Brodmann's,Brodmanns Area 8,Cortex, Frontal,Eye Field, Frontal,Eye Field, Supplementary,Eye Fields, Frontal,Frontal Cortices,Frontal Eye Field,Frontal Lobes,Lobe, Frontal,Supplementary Eye Fields
D006212 Hallucinations Subjectively experienced sensations in the absence of an appropriate stimulus, but which are regarded by the individual as real. They may be of organic origin or associated with MENTAL DISORDERS. Hallucination of Body Sensation,Hallucinations, Auditory,Hallucinations, Dissociative,Hallucinations, Elementary,Hallucinations, Formed, of People,Hallucinations, Gustatory,Hallucinations, Hypnagogic,Hallucinations, Hypnapompic,Hallucinations, Internal Body Sensation,Hallucinations, Kinesthetic,Hallucinations, Mood Congruent,Hallucinations, Mood Incongruent,Hallucinations, Olfactory,Hallucinations, Organic,Hallucinations, Reflex,Hallucinations, Sensory,Hallucinations, Somatic,Hallucinations, Tactile,Hallucinations, Verbal Auditory,Hallucinations, Visual,Hallucinations, Visual, Formed,Hallucinations, Visual, Unformed,Auditory Hallucination,Auditory Hallucination, Verbal,Auditory Hallucinations,Auditory Hallucinations, Verbal,Body Sensation Hallucination,Body Sensation Hallucinations,Dissociative Hallucination,Dissociative Hallucinations,Elementary Hallucination,Elementary Hallucinations,Gustatory Hallucination,Gustatory Hallucinations,Hallucination,Hallucination, Auditory,Hallucination, Dissociative,Hallucination, Elementary,Hallucination, Gustatory,Hallucination, Hypnagogic,Hallucination, Hypnapompic,Hallucination, Kinesthetic,Hallucination, Mood Congruent,Hallucination, Mood Incongruent,Hallucination, Olfactory,Hallucination, Organic,Hallucination, Reflex,Hallucination, Sensory,Hallucination, Somatic,Hallucination, Tactile,Hallucination, Verbal Auditory,Hallucination, Visual,Hypnagogic Hallucination,Hypnagogic Hallucinations,Hypnapompic Hallucination,Hypnapompic Hallucinations,Kinesthetic Hallucination,Kinesthetic Hallucinations,Mood Congruent Hallucination,Mood Congruent Hallucinations,Mood Incongruent Hallucination,Mood Incongruent Hallucinations,Olfactory Hallucination,Olfactory Hallucinations,Organic Hallucination,Organic Hallucinations,Reflex Hallucination,Reflex Hallucinations,Sensory Hallucination,Sensory Hallucinations,Somatic Hallucination,Somatic Hallucinations,Tactile Hallucination,Tactile Hallucinations,Verbal Auditory Hallucination,Verbal Auditory Hallucinations,Visual Hallucination,Visual Hallucinations
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006948 Hyperkinesis Excessive movement of muscles of the body as a whole, which may be associated with organic or psychological disorders. Hyperactivity, Motor,Hyperkinesia,Hyperkinesia, Generalized,Hyperkinetic Movements,Generalized Hyperkinesia,Generalized Hyperkinesias,Hyperkinesias, Generalized,Hyperkinetic Movement,Motor Hyperactivity,Movement, Hyperkinetic,Movements, Hyperkinetic
D000203 Activities of Daily Living The performance of the basic activities of self care, such as dressing, ambulation, or eating. ADL,Chronic Limitation of Activity,Limitation of Activity, Chronic,Activities, Daily Living,Activity, Daily Living,Daily Living Activities,Daily Living Activity,Living Activities, Daily,Living Activity, Daily

Related Publications

C A Mangone
June 1989, Journal of the American Geriatrics Society,
C A Mangone
January 1996, Journal of geriatric psychiatry and neurology,
Copied contents to your clipboard!