77
Views
14
CrossRef citations to date
0
Altmetric
Research Article

Neuropathology of amyotrophic lateral sclerosis with extra-motor system degeneration: Characteristics and differences in the molecular pathology between ALS with dementia and Guamanian ALS

Pages 97-104 | Published online: 10 Jul 2009

  • Kalo S, Oda M, Hayashi H, Kawata A, Shimizu T. Participation of the limbic system and its associated areas in the dementia of amyotrophic lateral sclerosis. J Neurol Sci 1994; 126: 62-69.
  • Knopman DS, Mastri AR, Frey WH 2, Sung JH, Rustan T. Dementia lacking distinctive histological features: a common non-Alzheimer degenerative dementia. Neurology 1990; 40: 251-256.
  • Mann DMA, South PW, Snowden JS, Neaiy D. Dementia of frontal lobe type: neuropathology and immunohistochemistiy. J Neurol Neurosurg Psychiatiy 1993; 56: 605-614.
  • Mitsuyama Y. Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. J Neurol Neurosurg Psychiatiy 1984; 47: 953-959.
  • Nakano I, Iwatsubo T, Hashizume Y, Mizutani T, Mannen T. Amyotrophic lateral sclerosis with dementia: lesions in the apical cortex and some deeper structures of the temporal lobes. Neuropathology 1992; 12: 69-77.
  • Nakano I. Temporal lobe lesions in amyotrophic lateral sclerosis with or without dementia: a neuropathological study. Neuropathology 1993; 13: 215-227.
  • Neary D, Snowden JS, Mann DMA, Northen B, Colliding PJ, Macdermott N. Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry 1990; 53: 23-32.
  • Wikstrom J, Paetau A, PaIo J, Sulkava R, Haltia M. Classic amyotrophic lateral sclerosis with dementia. Arch Neurol 1982; 39: 681-683.
  • Cooper PN, Jackson M, Lennox G, Lowe J, Mann DM. Tau, ubiquitin, and alpha B-crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia. Arch Neurol 1995; 52: 1011-1015.
  • Leigh PN. Pathogenic mechanisms in amyotrophic lateral sclerosis and other motor neuron disorders. In: Calne DB, ed. Neurodegenerative diseases. Philadelphia, WB Saunders, 1994: 474-477.
  • Okamato K, Murakami N, Kusaka H, et al. Ubiquitinpositive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease. J Neurol 1992; 239: 426-430.
  • Wightman G, Anderson VER, Martin J, et al. Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia. Neurosci Lett 1992; 139: 269-274.
  • Kawashima T, Kikuchi H, Takita M et al. Skein-like inclusions in the neostriatum from a case of amyotrophic lateral sclerosis with dementia. Acta Neuropathol 1998; 96: 541-545.
  • Konagaya M, Sakai M, Matsuoka Y, Kanagaya Y, Hashizume Y. Upper motor neuron predominant degeneration with frontal and temporal lobe atrophy. Acta Neuropathol 1998;96: 532-536.
  • Okamoto K, Hirai S, Yamazaki T, Sun X, Nakazato Y. New ubiquitin-positive intraneuronal inclusions in the extramotor cortices in patients with amyotrophic lateral sclerosis. Neurosci Lett 1991; 129: 233-236.
  • Bergmann M, Kuchelmeister K, Schmid KW, Kretzschmar HA, Schroder R. Different variants of frontotemporal dementia: a neuropathological and immunohistological study. Acta Neuropathol 1996; 92: 170-179.
  • Jackson M, Lennox G, Lowe J. Motor neuron disease-inclusion dementia. Neurodegeneration 1996; 5: 339-350.
  • Nakayama H, Arai N, Andou J, Akashi T. Three autopsied cases of frontotemporal dementia with pyramidal tract lesion (in Japanese). Neuropathology 1999; 19: 93.
  • The Lund and Manchester Groups. Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 1994; 57: 416-418.
  • Abrahams S, Goldstein LH, Kew JJ, Brook DJ, Lloyd CM, Frith CD, Leigh PN. Frontal lobe dysfunction in amyotrophic lateral sclerosis. A PET study. Brain 1996; 119: 2105-2120.
  • Brody JA, Hirano A, Scott RM. Recent neuropathologic observations in amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam. Neurology 1971; 21: 528-536.
  • Hirano A, Malamud N, Elizan TS, Kurland LT. Amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam. Further pathologic studies. Arch Neurol 1966; 15: 35-51.
  • Malamud N, Hirano A, Kurland LT. Pathoanatomic changes in amyotrophic lateral sclerosis on Guam. Special reference to the occurrence of neurofibrillary changes. Arch Neurol 1961; 5: 401-415.
  • Oyanagi K, Makifuchi T, Ohtoh T, et al. Amyotrophic lateral sclerosis of Guam: the nature of the neuropathological findings. Acta Neuropathol 1994; 88: 405-412.
  • Chen L. Neurofibrillary change on Guam. Arch Neurol 1981; 38: 16-18.
  • Hirano A, Malamud N, Kurland LT. Parkinsonism-dementia complex, an endemic disease on the island of Guam. 2. Pathological features. Brain 1961; 84: 662-679.
  • Hirano A, Llena JF. Neuropathological features of parkinsonism-dementia complex on Guam: reappraisal and comparative study with Alzheimer's disease and Parkinson's disease. In: Zimmerman HM, ed. Progress in neuropathology, Vol. 6. New York, Raven Press, 1986: 17-31.
  • Kato S, Oda M, Hayashi H. Neuropathology in amyotrophic lateral sclerosis patients on respirators: uniformity and diversity in 13 cases. Neuropathology 1993; 13: 229-236.
  • Kato S, Hayashi H, Oda M, et al. Neuropathology in sporadic ALS patients on respirators. In: Nakano 1Iand Hirano A, eds. Amyotrophic lateral sclerosis: progress and perspectives in basic research and clinical application. Amsterdam: Elsevier Science, 1996: 66-77.
  • Mizutani T, Sakamaki S, Tsuchiya N, et al. Amyotrophic lateral sclerosis with ophthalmoplegia and multisystem degeneration in patients on long-term use of respirators. Acta Neuropathol 1992; 84: 372-377.
  • Sasaki S, Tsutsumi Y, Yamane K, Sakuma H, Maruyama S. Sporadic amyotrophic lateral sclerosis with extensive neurological involvement. Acta Neuropathol 1992; 84: 211-215.
  • Hirano A. Neuropathology of familial amyotrophic lateral sclerosis patients with Superoxide dismutase 1 gene mutation. Neuropathology 1998; 18: 363-369.
  • Kato S, Shimoda M, Watanabe Y, Nakashima K, Takahashi K, Ohama E. Familial amyotrophic lateral sclerosis with a two base pair deletion in Superoxide dismutase 1 gene: multisystem degeneration with intracytoplasmic hyaline inclusion in astrocytes. J Neuropathol Exp Neurol 1996; 55: 1089-1101.
  • Kato S, Oda M, Tanabe H. Diminution of dopaminergic neurons in the substantia nigra of sporadic amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 1993; 19: 300-304.
  • Engel WK, Kurland ET, Klatzo I. An inherited disease similar to amyotrophic lateral sclerosis with a pattern of posterior column involvement An intermediate form? Brain 1959; 82: 203-220.
  • Hirano A, Kurland ET, Sayre GP. Familial amyotrophic lateral sclerosis: a subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells. Arch Neurol 1967; 16: 232-243.
  • Kato S, Hirano A. Involvement of the brain stem reticular formation in familial amyotrophic lateral sclerosis. Clin Neuropathol 1992; 11: 41-44.
  • Tanaka J, Nakamura H, Tabuchi Y, Takahashi K. Familial amyotrophic lateral sclerosis: features of multisystem degeneration. Acta Neuropathol 1984; 64: 22-29.
  • Yashida M, Okuda S, Murakami N, Hashizume Y, Sobue G. Two siblings of familial amyotrophic lateral sclerosis with multisystemic degeneration characterized by mild involvement of the middle root zone of the posterior column, Clarke's nuclei and spinocerebellar tract. Clin Neurol 1995; 35: 589-590.
  • lnce PG, Shaw PJ, Slade JY, Jones C, Hudgson P. Familial amyotrophic lateral sclerosis with a mutation in exon 4 of the Cu/Zn Superoxide dismutase gene: pathological and immunocytochemical changes. Acta Neuropathol 1996; 92: 395-403.
  • lnce PG, Tomkins J, Slade JY, Thatcher NM, Shaw PJ. Amyotrophic lateral sclerosis associated with genetic abnormalities in the gene encoding Cu/Zn Superoxide dismutase: molecular pathology of five new cases, and comparison with previous reports and 73 sporadic cases of AES. J Neuropathol Exp Neurol 1998; 57: 895-904.
  • Shibata N, Hirano A, Kobayashi M, et al. Intense Superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement. J Neuropathol Exp Neurol 1996; 55: 481-490.
  • Kato S, Kawata A, Oda M, Arai N, Komori T, Tanabe T. Absence of SODl gene abnormalities in familial amyotrophic lateral sclerosis with posterior column involvement without Eewy-body-like hyaline inclusions. Acta Neuropathol 1996; 92: 528-533.
  • Hudson AJ. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain 1981; 104: 217-247.
  • Finlayson MH, Guberman A, Martin JB. Cerebral lesions in familial amyotrophic lateral sclerosis and dementia. Acta Neuropathol 1973; 25: 237-246.
  • Pinsky L, Finlayson MH, Libman I; Scott BH. Familial amyotrophic lateral sclerosis with dementia: a second Canadian family. Clin Genet 1975; 7: 186-191.
  • Robertson EE. Progressive bulbar paralysis showing heredofamilial incidence and intellectual impairment. Arch Neurol Pysch 1953; 69: 197-207.
  • Shaw CE, Enayat ZE, Powell JF, et al. Familial amyotrophic lateral sclerosis. Molecular pathology of a patient with a SODI mutation. Neurology 1997; 49: 1612-1616.
  • Shimizu T, Yamaguchi S, Kato S, et al. Autopsy of a case of sporadic amyotrophic lateral sclerosis with autonomie failure (in Japanese). Neuropathology 1998; 18: 275.
  • Rouleau CA, Clark AW, Rooke K, et al. SODl mutation is associated with accumulation of neurofilaments in amyotrophic lateral sclerosis. Ann Neurol 1996; 39: 128-131.
  • Siddique T, Hentati A. Familial amyotrophic lateral sclerosis. Clin Neurosci 1995; 3: 338-347.
  • Poorkaj P, Bird TD, Wijsman E, et al. Tau is a candidate gene for chromosome 17 frontotemporal dementia. Ann Neurol 1998; 43: 815-825.
  • Jennissen HP. Ubiquitin and the enigma of intracellular protein degradation. Eur J Biochem 1995; 231: 159-163.
  • Rechsteiner M. Ubiquitin-mediated pathways for intracellular proteolysis. Annu Rev Cell Biol 1987; 3: 1-30.
  • Mori H, Kondo J, Ihara Y. Ubiquitin is a component of paired helical filaments in Alzheimer's disease. Science 1987; 235: 1641-1644.
  • Kuzuhara S, Mori H, Izumiyama N, et al. Lewy bodies are ubiquitinated. A light and electron microscopic immunocytochemical study. Acta Neuropathol 1988; 75: 345-353.
  • Love S, Saitoh T, Qijada S, et al. AIz50, ubiquitin and tau immunoreactivity of neurofibrillary tangles, Pick bodies and Eewy bodies. J Neuropathol Exp Neurol 1988; 47: 393-405.
  • Eeigh PN, Anderton A, Dodson A, et al. Ubiquitin deposits in anterior horn cells in motor neurone disease. Neurosci Eett 1988; 93: 197-203.
  • Mizusawa H, Nakamura H, Wakayama I, Yen SH, Hirano A. Skein-like inclusions in the anterior horn cells in motor neuron disease. J Neurol Sci 1991; 105: 14-21.
  • Lowe J. New pathological findings in amyotrophic lateral sclerosis. J Neurol Sci 1994; 124 (Suppl): 38-51.
  • Chou SM. Pathognomy of intraneuronal inclusions in ALS. In: Tsubaki Y, Toyokura Y, eds. Amyotrophic lateral sclerosis. Tokyo, University of Tokyo Press, 1978: 135-176.
  • Kato T, Katagiri T, Hirano A, et al. Eewy body-like hyaline inclusions in sporadic lower motor neuron disease are ubiquitinated. Acta Neuropathol 1989; 77: 391-396.
  • Kusaka H, Matsumoto S, lmai T. Granulofilamentous profiles in lower motor neurons: a sporadic case of amyotrophic lateral sclerosis with many Lewy body-like inclusions. Clin Neuropathol 1992; 11: 20-24.
  • Sasaki S, Yamane K, Sakuma H, et al. Sporadic motor neuron disease with Lewy body-like inclusion. Acta Neuropathol 1989; 78: 555-560.
  • Murayama S, Mori H, Ihara Y, Bouldin TW, Suzuki K, Tomonaga M. lmmunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis. Ann Neurol 1990; 27: 137-148.
  • Matsumoto S, Goto S, Kusaka H, et al. Ubiquitin-positive inclusion in anterior horn cells in subgroups of motor neuron diseases: a comparative study of adult-onset amyotrophic lateral sclerosis, juvenile amyotrophic lateral sclerosis and Werdnig-Hoffmann disease. J Neurol Sci 1993; 115: 208-213.
  • Matsumoto S, Hirano A, Goto S. Ubiquitin-immunoreactive filamentous inclusions in anterior horn cells of Guamanian and non-Guamanian amyotrophic lateral sclerosis. Acta Neuropathol 1990; 80: 233-238.
  • Suenaga T, Matsushima H, Nakamura S, et al. Ubiquitinimmunoreactive inclusions in anterior horn cells and hypoglossal neurons in a case with Joseph's disease. Acta Neuropathol 1993; 85: 341-344.
  • Mizusawa H, Hirano A, Yen SH. Anterior horn cell inclusions in familial amyotrophic lateral sclerosis contain ubiquitin and phosphorylated neurofilament epitopes. Neuropathology 1991; 11: 11-20.
  • Murayama S, Ohkawa Y, Mori H, et al. lmmunocytochemical and ultrastructural study of Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis. Acta Neuropathol 1989; 78: 143-152.
  • Sasaki S, Ohsawa Y, Yamane K, et al. Familial amyotrophic lateral sclerosis with widespread vacuolation and hyaline inclusions. Neurology 1998; 51: 871-873.
  • Ito H, Goto S, Hirano A, Yen S-H. Immunohistochemical study of the hippocampus in parkinsonism-dementia complex on Guam. J Geriat Psych Neurol 1991; 4: 134-142.
  • Hirano A, Dembitzer HM, Kurland LT, Zimmerman HM. The fine structure of some intraganglionic alterations: neurofibrillary tangles, granulovacuolar bodies and "rod-like" structures as seen in Guam amyotrophic lateral sclerosis and parkinsonism-dementia complex. J Neuropathol Exp Neurol 1968; 27: 167-182.
  • Oyanagi K, Makifuchi T, Ohtoh T, Chen KM, Gajdusek DC, Chase TN. Distinct pathological features of the gallyas- and tau-positive glia in the Parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam. J Neuropathol Exp Neurol 1997; 56: 308-316.
  • Arima K, Ogawa M, Sunohara N, Nishio T, Shimomura Y, Hirai S, Eto K. lmmunohistochemical and ultrastructural characterization of ubiquitinated eosinophilic fibrillary neuronal inclusions in sporadic amyotrophic lateral sclerosis. Acta Neuropathol 1998; 96: 75-85.
  • Hirano A, Kurland LT, Krooth RS, Eessell S. Parkinsonismdementia complex, an endemic disease on the island of Guam. 1. Clinical features. Brain 1961; 84: 642-661.
  • Leigh PN, Whitewell H, Garofaro O et al. Ubiquitinimmunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis: morphology, distribution, and specificity. Brain 1991; 114: 775-788.
  • Matsumoto S, Hirano S, Goto S. Spinal cord neurofibrillary tangles of Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia complex: an immunohistochemical study. Neurology 1990; 40: 975-979.
  • Umahara T, Hirano A, Kato S, Shibata N, Yen SH. Demonstration of neurofibrillary tangles and neuropil thread-like structures in spinal cord white matter in parkinsonism-dementia complex on Guam and in Guamanian amyotrophic lateral sclerosis. Acta Neuropathol 1994; 88: 180-184.
  • Ikemoto A, Hirano A, Akiguchi I, Kimura J. Comparative study of ubiquitin immunoreactivity of hippocampal granular cells in amyotrophic lateral sclerosis with dementia, Guamanian amyotrophic lateral sclerosis and Guamanian parkinsonism-dementia complex. Acta Neuropathol 1997; 93: 265-270.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.