{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T04:52:52Z","timestamp":1791089572045,"version":"4.1.0"},"reference-count":62,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,2,26]],"date-time":"2004-02-26T00:00:00Z","timestamp":1077753600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2004,3]]},"abstract":"<jats:p>Amyotrophic lateral sclerosis with dementia (ALS\u2010D) is a non\u2010Alzheimer\u2010type dementia characterized by both frontotemporal degeneration and motor neuron disease and marked by ubiquitin\u2010positive, tau\u2010 and \u03b1\u2010synuclein\u2010negative intraneuronal inclusions and dystrophic neurites. New neuropathological diagnostic criteria for ALS\u2010D are proposed on the basis of the present investigation of 28 autopsy cases. Clinical features included those of typical ALS\u2010D, primary lateral sclerosis, atypical ALS with frontotemporal atrophy and atypical Pick's disease without Pick's bodies. Macroscopically anterior frontotemporal atrophy was observed involving or not involving the precentral gyrus. Microscopically non\u2010specific neuronal loss and gliosis with spongiosis were seen, particularly in superficial layers II and III of the frontotemporal cortices. Diffuse fibrous gliosis was seen in the frontotemporal white matter. The substantia nigra and amygdala showed neuronal loss and gliosis. In all 28 cases, degeneration of both the lower and upper motor neuron systems, consistent with classic sporadic ALS, was present. The distribution and degree of degenerative frontotemporal lesions and motor neuron disturbance were of various patterns. Ubiquitin\u2010positive and tau\u2010 and \u03b1\u2010synuclein negative intraneuronal inclusions and dystrophic neurites in extramotor cortices were observed in all cases. Furthermore, ubiquitin\u2010positive inclusions in lower motor neurons were found in all cases. The distribution pattern and density differed between neuronal inclusions and dystrophic neurites and correlated with clinicopathological phenotypes. Therefore, the ALS\u2010D spectrum may be broader than that previously recognized, extending to primary lateral sclerosis, atypical ALS and to atypical Pick's disease without Pick bodies. Further investigation is needed to determine the characteristics of the ubiquitinated component in ALS\u2010D.<\/jats:p>","DOI":"10.1111\/j.1440-1789.2003.00544.x","type":"journal-article","created":{"date-parts":[[2004,2,26]],"date-time":"2004-02-26T14:40:48Z","timestamp":1077806448000},"page":"87-102","source":"Crossref","is-referenced-by-count":81,"title":["Amyotrophic lateral sclerosis with dementia: The clinicopathological spectrum"],"prefix":"10.1111","volume":"24","author":[{"given":"Mari","family":"Yoshida","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,2,26]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004150170199"},{"key":"e_1_2_7_3_2","first-page":"529","article-title":"Amyotrophic lateral sclerosis with organic dementia: Report of a case","volume":"4","author":"Yuasa R","year":"1964","journal-title":"Rinsho Shinkeigaku"},{"key":"e_1_2_7_4_2","first-page":"569","article-title":"Amyotrophic lateral sclerosis with dementia","volume":"10","author":"Yuasa R","year":"1970","journal-title":"Rinsho Shinkeigaku"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1979.00500450086021"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.47.9.953"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0167-4943(87)90026-4"},{"key":"e_1_2_7_8_2","first-page":"1193","article-title":"A clinicopathological study on 13 cases of motor neuron disease with dementia","volume":"32","author":"Yoshida M","year":"1992","journal-title":"Rinsho Shinkeigaku"},{"key":"e_1_2_7_9_2","first-page":"1560","article-title":"Reappraisal of amyotrophic lateral sclerosis with dementia","volume":"35","author":"Murakami N","year":"1995","journal-title":"Rinsho Shinkeigaku"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(91)90469-A"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(92)90569-S"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00856806"},{"key":"e_1_2_7_13_2","first-page":"69","article-title":"Amyotrophic lateral sclerosis with dementia\u2010lesions in the apical cortex and some deeper structures of the temporal lobes","volume":"12","author":"Nakano I","year":"1992","journal-title":"Neuropathology"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.1993.tb00200.x"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(94)90095-7"},{"key":"e_1_2_7_16_2","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1007\/s00401-002-0620-y","article-title":"The relationship between extramotor ubiquitin\u2010immunoreactive neuronal inclusions and dementia in motor neuron disease","volume":"105","author":"Mackenzie IRA","year":"2003","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.2003.tb00002.x"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1789.2002.00451.x"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.58.11.1803"},{"key":"e_1_2_7_20_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10048-002-0140-x"},{"key":"e_1_2_7_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(98)00168-3"},{"key":"e_1_2_7_22_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-001-0513-5"},{"key":"e_1_2_7_23_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.53.1.23"},{"key":"e_1_2_7_24_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.57.4.416"},{"key":"e_1_2_7_25_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.40.2.251"},{"key":"e_1_2_7_26_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.1995.tb01095.x"},{"key":"e_1_2_7_27_2","doi-asserted-by":"publisher","DOI":"10.1006\/neur.1996.0046"},{"key":"e_1_2_7_28_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00308809"},{"key":"e_1_2_7_29_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.4.479"},{"key":"e_1_2_7_30_2","first-page":"A62","article-title":"An autopsy case of amyotrophic lateral sclerosis with dementia after 7.5 years of artificial ventilation","volume":"19","author":"Horimoto Y","year":"1999","journal-title":"Neuropathology"},{"key":"e_1_2_7_31_2","first-page":"A40","article-title":"An autopsy case of ALS presenting marked degeneration of precentral gyrus, putamen and globus pallidus","volume":"23","author":"Sone M","year":"2003","journal-title":"Neuropathology"},{"key":"e_1_2_7_32_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050930"},{"key":"e_1_2_7_33_2","first-page":"A40","article-title":"Sporadic motor neuron disease with marked frontotemporal atrophy: An autopsied case mimicking primary lateral sclerosis","volume":"23","author":"Koga H","year":"2003","journal-title":"Neuropathology"},{"key":"e_1_2_7_34_2","first-page":"A40","article-title":"An autopsy case of ALS wearing respirator for 10 years and 2 months","volume":"23","author":"Yasui K","year":"2003","journal-title":"Neuropathology"},{"key":"e_1_2_7_35_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00294323"},{"key":"e_1_2_7_36_2","first-page":"A39","article-title":"An autopsy case of frontotemporal dementia showing pyramidal tract degeneration with ubiquitinated intraneuronal inclusions and neurites","volume":"23","author":"Banno H","year":"2003","journal-title":"Neuropathology"},{"key":"e_1_2_7_37_2","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1007\/s00401-002-0543-7","article-title":"Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases","volume":"104","author":"Tsuchiya K","year":"2002","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_38_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2990.2002.00384.x"},{"key":"e_1_2_7_39_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(88)90081-X"},{"key":"e_1_2_7_40_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(88)90296-0"},{"key":"e_1_2_7_41_2","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1007\/s004010000291","article-title":"Bunina body in frontal lobe dementia without clinical manifestations of motor neuron disease","volume":"101","author":"Uchihara T","year":"2001","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_42_2","first-page":"66","volume-title":"Amyotrophicllateral Sclerosis: Progress and Perspectives in Basic Research and Clinical Application","author":"Kato S","year":"1996"},{"key":"e_1_2_7_43_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.36.10.1323"},{"key":"e_1_2_7_44_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410330210"},{"key":"e_1_2_7_45_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00296365"},{"key":"e_1_2_7_46_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(96)00315-2"},{"key":"e_1_2_7_47_2","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1007\/s004010000336","article-title":"Atypical amyotrophic lateral sclerosis with dementia mimicking frontal Pick's disease: a report of an aytopsy case with a clinical course of 15 years","volume":"101","author":"Tsuchiya K","year":"2001","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_48_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1789.1999.00202.x"},{"key":"e_1_2_7_49_2","first-page":"A18","article-title":"An autopsy case of amyotrophic lateral sclerosis with upper motor neuron (UMN) predominant degeneration presenting slowly progressive aphasia: a subtype of ALS with dementia (ALS\u2010D)?","volume":"20","author":"Yamazaki M","year":"2000","journal-title":"Neuropathology"},{"key":"e_1_2_7_50_2","first-page":"A12","article-title":"A case of frontotemporal atrophy with ubiquitin inclusions presenting with upper motor neuron signs at onset followed by extrapyramidal signs and dementia","volume":"22","author":"Mochizuki A","year":"2002","journal-title":"Neuropathology"},{"key":"e_1_2_7_51_2","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1007\/s00401-003-0687-0","article-title":"Primary lateral sclerosis: a rare upper\u2010motor\u2010predominant form of amyotrophic lateral sclerosis often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions? Report of an autopsy case and a review of the literature","volume":"105","author":"Tan CF","year":"2003","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_52_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050505"},{"key":"e_1_2_7_53_2","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1007\/s00401-002-0611-z","article-title":"Frontotemporal dementia with cerebral intraneuronal ubiquitin\u2010positive inclusions but lacking lower motor neuron involvement","volume":"105","author":"Yaguchi M","year":"2003","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_54_2","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1007\/s00401-003-0696-z","article-title":"Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease\u2010inclusion dementia","volume":"106","author":"Toyoshima Y","year":"2003","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_55_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.1.115"},{"key":"e_1_2_7_56_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.2.267"},{"key":"e_1_2_7_57_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8257(199907)14:4<674::AID-MDS1019>3.0.CO;2-X"},{"key":"e_1_2_7_58_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010100457"},{"key":"e_1_2_7_59_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2990.1998.00108.x"},{"key":"e_1_2_7_60_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050555"},{"key":"e_1_2_7_61_2","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1007\/s004010000346","article-title":"Frontotemporal dementia with ubiquitinated cytoplasmic and intranuclear inclusions","volume":"102","author":"Woulfe J","year":"2001","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_62_2","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1007\/s00401-003-0678-1","article-title":"Neuronal intranuclear inclusions distinguishing familial FTD\u2010MND type from sporadic cases","volume":"105","author":"Mackenzie IRA","year":"2003","journal-title":"Acta Neuropathol (Berl)"},{"key":"e_1_2_7_63_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(03)00216-7"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1440-1789.2003.00544.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1440-1789.2003.00544.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T17:43:25Z","timestamp":1696959805000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1440-1789.2003.00544.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,2,26]]},"references-count":62,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2004,3]]}},"alternative-id":["10.1111\/j.1440-1789.2003.00544.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1440-1789.2003.00544.x","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,2,26]]}}}