A patient presenting with the characteristic clinical features of the dementia of Pick's type is described, in whom neuropathological examination of brain biopsy material revealed atypical features, including extensive subcotical gliosis with mild cortical neuronal loss and without any neuronal cytoskeletal inclusions (Pick bodies, neurofibrillary tangles, and Lewy bodies) and amyloid deposits (senile plaques). And she has the suggestive family history of the same clinical features in her two brothers. So, the clinical and pathological features are discussed with particular reference to typical Alzheimer's disease and Pick's disease, and it is proposed that the case should be classified as familial progressive subcortical gliosis.
Alzheimer Disease ; Biopsy ; Brain ; Dementia ; Gliosis* ; Humans ; Neurofibrillary Tangles ; Neurons ; Pick Disease of the Brain ; Plaque, Amyloid ; Siblings

Niemann-Pick disease is a storage disease characterized by accumulation of sphingomyelin and other lipids, mainly in the reticuloendothelial system. We experienced a case of type A Niemann-Pick disease in a 18-month-old male infant. He showed dyspnea, marked hepatosplenomegaly and developmental retardation. Fundoscopic examination revealed cherry red spots in both macula. Bone marrow aspirates showed characteristic foam cells. Autopsy finding revealed that liver, spleen, lung, lymph node and brain were involved. Reticular infiltration was shown on chest X-ray. We reported a case of type A Niemann-Pick disease with a brief review of the related literature.
Autopsy ; Bone Marrow ; Brain ; Dyspnea ; Foam Cells ; Humans ; Infant ; Liver ; Lung ; Lymph Nodes ; Male ; Mononuclear Phagocyte System ; Niemann-Pick Disease, Type A* ; Niemann-Pick Diseases ; Prunus ; Spleen ; Thorax

Frontotemporal lobe dementia have been underevaluated because of various clinical features, changing diagnostic criteria, and indifference of clinicians. It is important that frontotemporal lobe dementia patient showing behavioral and lingual problems should be early diagnosed and treated. Because frontotemporal lobe dementia patients often confused with Alzheimer's disease, senile depression, schizophrenia, drug abuse. We have presented a case of frontotemporal lobe dementia. He had typical clinical history and symptoms which deserve to be considered frontotemporal lobe dementia. He showed appropriate findings of frontotemporal lobe dementia in the neuropsychological tests and brain magnetic resonance imaging and single photon emission computed tomography. This case is thought to be helpful for clinicians to give attention to early diagnosis and appropriate treatment of frontotemporal lobe dementia.
Alzheimer Disease ; Brain ; Depression ; Early Diagnosis ; Frontotemporal Dementia* ; Humans ; Magnetic Resonance Imaging ; Neuropsychological Tests ; Pick Disease of the Brain ; Schizophrenia ; Substance-Related Disorders ; Tomography, Emission-Computed, Single-Photon

Dementias can be calssified into cortical, subcortical, cortical-subcortical and multifocal ones based on the major pathological distribution within the brain. The literatures of recent knowledge about clinical features of other dementias than Alzheimer's and vascular ones, which were most frequently experienced by many clinicians were reviewed. That is, cortical dementias such as Pick's disease, frontal lobe type dementia and non-Alzheimer's type lobar atrophy including fronto-temporal dementia, progressive dysphasia, fronto-temporal dementia with motor neuron disease, and alcohol-related dementia were reviewed. Subcortical dementias such as dementias accompanying Parkinson's disease, Huntington's disease and progressive supranuclear palsy, and cortical-subcortical dementias such as Lewy body dementiaq and cortical-basal degeneration were also reviewed. As multifocal dementias, prion dementias including KUru, Creutzfeldt-Jakob disease, fatal familial insomnia and Gerstmann-Strussler-Sheinker syndrone, and AIDS dementia were also reviewed.
Aphasia ; Atrophy ; Brain ; Creutzfeldt-Jakob Syndrome ; Dementia* ; Frontal Lobe ; Frontotemporal Dementia ; Huntington Disease ; Insomnia, Fatal Familial ; Kuru ; Lewy Bodies ; Lewy Body Disease ; Motor Neuron Disease ; Parkinson Disease ; Pick Disease of the Brain ; Supranuclear Palsy, Progressive

Animals ; Brain ; metabolism ; pathology ; Calcium ; metabolism ; Cholesterol ; metabolism ; Glycosphingolipids ; metabolism ; Humans ; Liver ; pathology ; Neurons ; metabolism ; pathology ; Niemann-Pick Disease, Type C ; diagnosis ; etiology ; metabolism ; pathology ; Sphingosine ; metabolism

Criticisms about amyloid cascade hypothesis of Alzheimer's disease(AD) are based on the findings, first, that the degree of dementia does not correlate with the number of plaques, and second, that the neurofibrillary tangle formation seems to predate plaque formation. In addition, neurofibrillary tangle counts correlate well with the degree of cognitive impairment. These findings suggest the independent importance of tau abnormality in AD research which is involved in the neurofibrillary tangle formation. Recently, tau pathology without amyloid deposits and mutations in tau protein gene were reported to be the major pathogenic mechanism in Pick's disease, progressive supranuclear palsy, corticobasal degeneration and FTDP-17(frontotemporal dementia and parkinsonism linked with chromosome 17). These data suggest that understanding the causes and consequences of tau dysfunction might give new clinical and therapeutic solutions to many known tauopathies.
Amyloid ; Dementia ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration ; Molecular Biology* ; Neurofibrillary Tangles ; Parkinsonian Disorders ; Pathology ; Pick Disease of the Brain ; Plaque, Amyloid ; Prednisolone ; Supranuclear Palsy, Progressive ; tau Proteins* ; Tauopathies

OBJECTIVETo characterize histopathologic features of non-Alzheimer type dementia.

METHODSBodian, Gallyas-Braak silver staining, tau and ubiquitin immunohistochemistry were applied in an analysis of 22 cases of autopsy-proven neurodegenerative dementia. Appearance, distribution and immunoreactivity of neuronal and glial inclusions in the brain were observed. The final histological diagnoses were made according to the pathological criteria for several types of common non-Alzheimer type dementia.

RESULTSAmong the 22 cases of neurodegenerative dementia, 12 cases were identified as non-Alzheimer type dementia, including Pick's disease (2 cases), progressive supranuclear palsy (3 cases) and corticobasal degeneration (3 cases), dementia with Lewy bodies (1 case), and Parkinson's disease (3 cases). Another 10 cases consisted of pure Alzheimer's disease (AD, 9 cases) and AD combined with argyrophilic grain disease (1 case). Characteristic neuronal and glial inclusions, such as classical and cortical Lewy body, Pick body, Globous NFTs, astrocytic plaque and tufted astrocyte, argyrophilic grain were found in the brains of non-Alzheimer type dementia. Classical and cortical Lewy bodies were not argyrophilic but were immunoreactive to ubiquitin. Pick bodies, Globous NFTs, astrocytic plaques, tufted astrocytes and argyrophilic grains were all argyrophilic. Pick bodies showed tau and ubiquitin immunoreactivity. However, Globous NFTs, astrocytic plaques, tufted astrocytes, and argyrophilic grains were reactive only to tau immunohistochemistry.

CONCLUSIONSFindings of characteristic neuronal and glial inclusions may help to differentiate non-Alzheimer type dementia from AD, and in conjunction with Gallyas-Braak staining and immunohistochemistry for tau and ubiquitin, to further define histopathologic subcategories of non-Alzheimer type dementia.

Aged ; Aged, 80 and over ; Brain ; pathology ; Dementia ; pathology ; Diagnosis, Differential ; Female ; Humans ; Lewy Body Disease ; pathology ; Male ; Neurodegenerative Diseases ; pathology ; Neurons ; pathology ; Parkinson Disease ; pathology ; Pick Disease of the Brain ; pathology ; Supranuclear Palsy, Progressive ; pathology

Frontotemporal dementia (FTD), formerly called Pick's disease, is a progressive dementia that is associated with focal atrophy of the frontal and/or temporal lobes. FTD has three major clinical subtypes ; 1) a frontal variant of frontotemporal dementia (fvFTD), 2) semantic dementia (SD), and 3) progressive nonfluent aphasia (PNFA). These different variants differ in their clinical symptoms, cognitive deficits, and affected brain regions. The insidious onset of personality changes and behavioral abnormalities is the most prominent feature of fvFTD. Poor insight, loss of personal and social awareness, and blunting of affect are common behavioral changes in fvFTD. The most common presenting complaint in SD involves language, and is often described as a loss of memory for words or a loss of word meaning. Patients with PNFA present with changes in fluency, pronunciation, or word finding difficulty. An accumulating body of evidence suggests that FTD overlaps with three other neurodegenerative diseases: motor neuron disease (MND), corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP). Treatment for FTD consists of behavioral and pharmacological approaches. Medications such as selective serotonin reuptake inhibitors, antipsychotics have used in FTD. Cholinesterase inhibitors do not consistently improve cognitive and behavioral symptoms of FTD. Further research should be directed at developing new therapeutic methods to improve the patients' symptoms.
Antipsychotic Agents ; Atrophy ; Behavioral Symptoms ; Brain ; Cholinesterase Inhibitors ; Dementia ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration ; Humans ; Memory ; Motor Neuron Disease ; Neurobehavioral Manifestations ; Pick Disease of the Brain ; Primary Progressive Nonfluent Aphasia ; Serotonin Uptake Inhibitors ; Supranuclear Palsy, Progressive ; Temporal Lobe

BACKGROUND AND SIGNIFICANCE: PPA is the clinical syndrome that reveals a marked, progressive loss of language functions over time with relative preservation of non-linguistic cognitive functions. Patients with this syndrome eventually develop a general dementia, but the natural history, neuropathology, and etiology are poorly understood. We report a patient who suffered from progressive aphasia unaccompanied with prominent cognitive deficits for 5 years. CASE: A 64-year-old man presented with an at least a 5-year history of a progressive language disorder. The initial symptoms were a disability to speak in complete sentences, word-finding difficulty, verbal hesitancy, and resultant social withdrawal. Over the next 5-years, his symptoms slowly worsened, but non-verbal cognitive functions such as memory, visuospatial skill, and daily living activities were preserved. Eventually, the patient was mutistic, incommunicable, and impaired in general intelligence parallel with personality and mood changes like emotional lability, impersistence, or agitation. The imaging of brain revealed generalized atrophy with more prominent changes in the left temporal lobe and the degree of atrophy progressed on the follow-up study 4 years later. The brain SPECT showed hypoperfusion in the left frontotemporal area. Initially, the patient scored 25 points with Mini-Mental State Examination but worsened to 3 points 4 years later. Language examination at the onset showed mildly decreased fluency, naming, and reading. Recently, our patient has become mutistic and alexic, but has preserved some comprehension. COMMENT: Non-dementic patients with progressive language disturbances as a result of the left focal temporal atrophy from 2-year of onset, have the possibility of being diagnosed with PPA, frontotemporal degeneration, Pick disease, or Alzheimer disease. But we suggest that characteristic isolated linguistic dysfunction with preserved cognitive function in PPA may be a distinct from other degenerative diseases of dementia.
Activities of Daily Living ; Alzheimer Disease ; Aphasia ; Aphasia, Primary Progressive* ; Atrophy ; Brain ; Comprehension ; Dementia ; Dihydroergotamine ; Follow-Up Studies ; Humans ; Intelligence ; Language Disorders ; Linguistics ; Memory ; Middle Aged ; Natural History ; Pick Disease of the Brain ; Temporal Lobe ; Tomography, Emission-Computed, Single-Photon

Pick's disease is a rare neurodegenerative disorder presenting cortical type of dementia. Pick's disease shows unique clinical and pathological features, that are due to a degeneration of fronto-temporal lobes of the cerebrum. The authors experienced a case of Pick's disease in a 58-year-old male patient who had dementia symptoms for five years. The patient showed compulsive behavior since five years ago. Memory decline started from four years ago and progressed. Brain CT disclosed lobar atrophy of the cerebral gyri in frontal and temporal lobes. He died of septicemia associated with aspiration pneumonia. At autopsy, both cerebral hemispheres showed marked encephalomalacia. The gyral atrophy was moderately severe in prefrontal and anterior temporal lobes. Coronal section disclosed moderate dilatation of the lateral ventricles. Microscopically, there were marked neuronal loss in prefrontal and anterior temporal cortices. Also noted were Pick's cells and Pick's body in occasional pyramidal cells preserved.
Atrophy ; Autopsy* ; Brain ; Cerebrum ; Compulsive Behavior ; Dementia ; Dilatation ; Encephalomalacia ; Humans ; Lateral Ventricles ; Male ; Memory ; Middle Aged ; Neurodegenerative Diseases ; Neurons ; Pick Disease of the Brain* ; Pneumonia, Aspiration ; Pyramidal Cells ; Sepsis ; Temporal Lobe