Frontotemporal lobar degeneration: Difference between revisions
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'''Frontotemporal lobar degeneration''' (FTLD), | '''Frontotemporal lobar degeneration''' (FTLD), also known as '''Pick’s Disease''', is a deterioration of the physical structure of the [[frontal lobe]] of the brain. There are three clinical syndromes that result from this deterioration, [[frontotemporal dementia]], [[progressive nonfluent aphasia]] and [[semantic dementia]]. These syndromes involve perception of self and others, social skills and language. Symptoms of this malady may include impaired social and personal conduct, blunted emotions and loss of insight, and language disorder that may include nonfluent spontaneous speech fluent, meaningless spontaneous speech, and impaired word meaning. Unlike '''Alzheimer’s disease''', FTLD patients’ memories remain intact and visuospatial skills are unimpaired. The causes are unknown although there are possible genetic precursors that indicate the disease is inherited. '''Genetic mutations''' of '''chromosome 17''' have been identified in some cases but this is not consistent in all cases of the disorder.<ref>[http://www.dcmsonline.org/jax-medicine/2000journals/February2000/ftld.htm Frontotemporal Lobar Degeneration] Short, Rodney A. (2000)</ref> | ||
==Clinical Criteria== | ==Clinical Criteria== | ||
Core clinical features supporting diagnostic criteria of the subtypes: | |||
Core clinical features supporting diagnostic criteria of the subtypes: | |||
'''Frontotemporal dementia''' | '''Frontotemporal dementia''' | ||
The most common clinical subtype of FTLD characterised by personality change and impaired social conduct in its initial stages | The most common clinical subtype of FTLD characterised by personality change and impaired social conduct in its initial stages | ||
*decline in social interpersonal conduct | *decline in social interpersonal conduct | ||
*impairment in regulation of personal conduct | *impairment in regulation of personal conduct | ||
*emotional blunting | *emotional blunting | ||
*loss of insight | *loss of insight | ||
Other common features | Other common features | ||
*disinhibition | *disinhibition | ||
*neglect of personal hygygiene, mental rigidity | *neglect of personal hygygiene, mental rigidity, | ||
*perseverative behaviour | *perseverative behaviour | ||
*voracious appetite | *voracious appetite | ||
*hyperorality | *hyperorality | ||
'''Progressive nonfluent aphasia''' | '''Progressive nonfluent aphasia''' | ||
Nonfluent spontaneous speech with at least one of the following: | Nonfluent spontaneous speech with at least one of the following: | ||
*agrammatism | *agrammatism: A form of aphasia. The ability to speak is impaired to different degrees. In less extreme cases, the patient speaks in telegraphic speech (simplistic sentence structure similar to a telegraph message) but in more profound extremes by speaking in short groups of words, usually using nouns, without any grammatical structure. However, the patient understands what others are saying and can respond appropriately. | ||
*phonemic paraphasias | *phonemic paraphasias, | ||
*anomia | *anomia | ||
'''Semantic dementia''' | '''Semantic dementia''' | ||
Language disorder characterized by | Language disorder characterized by | ||
*progressive, fluent, empty spontaneous speech | *progressive, fluent, empty spontaneous speech | ||
*loss of word meaning, manifest by impaired naming and comprehension | *loss of word meaning, manifest by impaired naming and comprehension | ||
*semantic paraphasias | *semantic paraphasias | ||
Perceptual disorder characterized by | Perceptual disorder characterized by | ||
*prosopagnosia | *prosopagnosia: impaired recognition of identity of familiar faces | ||
*associative agnosia | *associative agnosia: impaired recognition of object identity | ||
Cognitive abilities that are preserved, i.e. not impaired | Cognitive abilities that are preserved, i.e. not impaired | ||
*perceptual matching and drawing reproduction | *perceptual matching and drawing reproduction | ||
*single-word repetition | *single-word repetition | ||
*ability to read aloud and write to dictation orthographically regular words | *ability to read aloud and write to dictation orthographically regular words | ||
==Neuropathology== | ==Neuropathology== | ||
| Line 72: | Line 48: | ||
==Histopathology== | ==Histopathology== | ||
'''Type 1''' | '''Type 1''' | ||
*prominent microvacuolar change in layer II and upper layer III of the cortical lamina | *prominent microvacuolar change in layer II and upper layer III of the cortical lamina | ||
*no specific histologic features meaning the absence of staining with immunohistochemical markers | *no specific histologic features meaning the absence of staining with immunohistochemical markers | ||
*in some cases neurons stain with an antibody to a B crystallin | *in some cases neurons stain with an antibody to a B crystallin | ||
'''Type 2''' | '''Type 2''' | ||
*severe astrocytic gliosis with or without ballooned neurons and inclusion bodies (Pick type or Pick's disease). | *severe astrocytic gliosis with or without ballooned neurons and inclusion bodies (Pick type or Pick's disease). | ||
*when present, ballooned neurons and inclusion bodies of the Pick type stain with antibodies directed against tau protein.<ref>[http://www.alzgmc.org/about_alz/glossary.htm] The tau protein is a protein composing neurofibrillary tangles found in degenerating nerve cells. The protein is a normal part of the internal structure of nerve cells. Tau protein is abnormally processed in Alzheimer’s.</ref> | *when present, ballooned neurons and inclusion bodies of the Pick type stain with antibodies directed against tau protein.<ref>[http://www.alzgmc.org/about_alz/glossary.htm] The tau protein is a protein composing neurofibrillary tangles found in degenerating nerve cells. The protein is a normal part of the internal structure of nerve cells. Tau protein is abnormally processed in Alzheimer’s.</ref> | ||
| Line 86: | Line 62: | ||
*tau pathology in addition to amyloid pathology and generalised atrophy | *tau pathology in addition to amyloid pathology and generalised atrophy | ||
'''corticobasal degeneration (CBD)''' | |||
*tau pathology | *tau pathology | ||
*may start as clinical syndromes similar to FTLD | *may start as clinical syndromes similar to FTLD | ||
''' | '''progressive supranuclear palsy (PSP)''' | ||
*tau pathology | *tau pathology | ||
*may start as clinical syndromes similar to FTLD | *may start as clinical syndromes similar to FTLD | ||
==Genetics== | ==Genetics== | ||
Revision as of 01:38, 6 October 2007
Frontotemporal lobar degeneration (FTLD), also known as Pick’s Disease, is a deterioration of the physical structure of the frontal lobe of the brain. There are three clinical syndromes that result from this deterioration, frontotemporal dementia, progressive nonfluent aphasia and semantic dementia. These syndromes involve perception of self and others, social skills and language. Symptoms of this malady may include impaired social and personal conduct, blunted emotions and loss of insight, and language disorder that may include nonfluent spontaneous speech fluent, meaningless spontaneous speech, and impaired word meaning. Unlike Alzheimer’s disease, FTLD patients’ memories remain intact and visuospatial skills are unimpaired. The causes are unknown although there are possible genetic precursors that indicate the disease is inherited. Genetic mutations of chromosome 17 have been identified in some cases but this is not consistent in all cases of the disorder.[1]
Clinical Criteria
Core clinical features supporting diagnostic criteria of the subtypes:
Frontotemporal dementia
The most common clinical subtype of FTLD characterised by personality change and impaired social conduct in its initial stages
- decline in social interpersonal conduct
- impairment in regulation of personal conduct
- emotional blunting
- loss of insight
Other common features
- disinhibition
- neglect of personal hygygiene, mental rigidity,
- perseverative behaviour
- voracious appetite
- hyperorality
Progressive nonfluent aphasia
Nonfluent spontaneous speech with at least one of the following:
- agrammatism: A form of aphasia. The ability to speak is impaired to different degrees. In less extreme cases, the patient speaks in telegraphic speech (simplistic sentence structure similar to a telegraph message) but in more profound extremes by speaking in short groups of words, usually using nouns, without any grammatical structure. However, the patient understands what others are saying and can respond appropriately.
- phonemic paraphasias,
- anomia
Semantic dementia
Language disorder characterized by
- progressive, fluent, empty spontaneous speech
- loss of word meaning, manifest by impaired naming and comprehension
- semantic paraphasias
Perceptual disorder characterized by
- prosopagnosia: impaired recognition of identity of familiar faces
- associative agnosia: impaired recognition of object identity
Cognitive abilities that are preserved, i.e. not impaired
- perceptual matching and drawing reproduction
- single-word repetition
- ability to read aloud and write to dictation orthographically regular words
Neuropathology
A common method of diagnosing FTLD patients is the use of scanning technology including MRI (magnetic resonance imaging), single photon emission computed tomography (SPECT) and PET (Positron Emission Tomography). These scans show that there is atrophy of certain areas of the brain, specifically a decrease in the size of the frontal and anterior temporal lobes.[2]
Histopathology
Type 1
- prominent microvacuolar change in layer II and upper layer III of the cortical lamina
- no specific histologic features meaning the absence of staining with immunohistochemical markers
- in some cases neurons stain with an antibody to a B crystallin
Type 2
- severe astrocytic gliosis with or without ballooned neurons and inclusion bodies (Pick type or Pick's disease).
- when present, ballooned neurons and inclusion bodies of the Pick type stain with antibodies directed against tau protein.[3]
- ballooned neurons also stain for neurofilament protein
Differential Diagnosis
Alzheimer’s disease
- tau pathology in addition to amyloid pathology and generalised atrophy
corticobasal degeneration (CBD)
- tau pathology
- may start as clinical syndromes similar to FTLD
progressive supranuclear palsy (PSP)
- tau pathology
- may start as clinical syndromes similar to FTLD
Genetics
There have been familial studies that strongly indicate the disease may be inherited. Gene mutations of tau protein on chromosome 17 have been found in some cases. However, there are numerous cases which have no evidence of this mutation. Similarly, the apolipoprotein E4 allele which is a risk factor in Alzheimer’s shows no association with FTLD.
References
- ↑ Frontotemporal Lobar Degeneration Short, Rodney A. (2000)
- ↑ Brain Imaging] Dr. Jonathan Kennedy Dementia Research Centre, Pick's Disease Support Group]]
- ↑ [1] The tau protein is a protein composing neurofibrillary tangles found in degenerating nerve cells. The protein is a normal part of the internal structure of nerve cells. Tau protein is abnormally processed in Alzheimer’s.