Progress in clinical neurosciences: The evidence for ALS as a multisystems disorder of limited phenotypic expression

Canadian Journal of Neurological Sciences

Volumn 28, Issue 4, 2001, Pages 283-298

  Strong, M J ^a  

^a LONDON HEALTH SCIENCES CENTRE   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

AZATHIOPRINE; BRAIN DERIVED NEUROTROPHIC FACTOR; BRANCHED CHAIN AMINO ACID; CASPASE; COPPER ZINC SUPEROXIDE DISMUTASE; CYCLOPHOSPHAMIDE; CYCLOSPORIN; CYTOCHROME C; CYTOSKELETON PROTEIN; DEXTROMETHORPHAN; GABAPENTIN; GLUTAMATE RECEPTOR ANTAGONIST; GLUTAMATE TRANSPORTER; GLUTAMIC ACID; HEXAMETHYLPROPYLENE AMINE OXIME TECHNETIUM TC 99M; HYDROXYL RADICAL; IMMUNOGLOBULIN; IMMUNOMODULATING AGENT; IOFETAMINE I 123; IONOTROPIC RECEPTOR; LAMOTRIGINE; METABOTROPIC RECEPTOR; NEUROTROPHIC FACTOR; PEROXYNITRITE; PREDNISONE; RECOMBINANT SOMATOMEDIN C; RILUZOLE; THREONINE; VERAPAMIL;

AGE; AMYOTROPHIC LATERAL SCLEROSIS; BRAIN MITOCHONDRION; CLINICAL TRIAL; COGNITIVE DEFECT; COMPUTER ASSISTED TOMOGRAPHY; FAMILIAL DISEASE; GLIA; HUMAN; MORTALITY; MOTONEURON; NEUROFILAMENT; NUCLEAR MAGNETIC RESONANCE IMAGING; OXIDATIVE STRESS; PHENOTYPE; PRIORITY JOURNAL; REVIEW; SINGLE PHOTON EMISSION COMPUTER TOMOGRAPHY; SURVIVAL TIME;

EID: 0035676020     PISSN: 03171671     EISSN: None     Source Type: Journal    
DOI: 10.1017/S0317167100001505     Document Type: Review

References (222)

1. Deux cas d'atrophie musculaire progressive avec lésions de la substance grise et des faisceaux antérolatéraux de la moelle épinière
2. Hereditary motor system diseases (chronic juvenile amyotrophic lateral sclerosis)
3. Aging issues in the workplace. Assisting workers who provide eldercare
4. Rising mortality from motoneuron disease in the USA, 1962-84
5. Increasing trend of ALS in France and elsewhere: Are the changes real?
6. Longitudinal gompertzian analysis of amyotrophic lateral sclerosis mortality in the U.S., 1977 - 1986: Evidence for an inherently susceptible population subset
7. Rising mortality from motor neurone disease in Sweden 1961 - 1990: The relative role of increased population life expectancy and environmental factors
8. The mortality of motor neuron disease in Sweden
9. Changes in the incidence of amyotrophic lateral sclerosis in Israel
10. Motor neuron disease in England and Wales, 1959 - 1979
11. The incidence of amyotrophic lateral sclerosis in southwestern Ontario, Canada
12. Exogenous neurotoxins
13. Amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam: Clinical, epidemiological and genetic patterns
14. Pacific paradigms of environmentally-induced neurological disorders: Clinical, epidemiological and molecular perspectives
15. Low-calcium, high aluminum diet-induced motor neuron pathology in cynomolgus monkeys
16. Epidemiological study of amyotrophic lateral sclerosis in Sardinia, Italy
17. Familial amyotrophic lateral sclerosis, 1850-1989: A statistical analysis of the world literature
18. Duration of amyotrophic lateral sclerosis is age dependent
19. Survival prediction in amyotrophic lateral sclerosis
20. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: A review
21. Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis
22. The relationship between abnormalities of cognitive function and cerebral activation in amyotrophic lateral sclerosis
23. Neuropsychological study of motor neuron disease
24. Neuropsychological dysfunctions in amyotrophic lateral sclerosis: Relation to motor disabilities
25. Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria
26. Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neuron disease-dementia-aphasia syndrome
27. Rapidly progressing aphasic dementia and motor neuron disease
28. Contributions of anterior cingulate cortex to behaviour
29. Cognition, language and speech in amyotrophic lateral sclerosis: A review
30. A prospective study of cognitive impairment in ALS
31. Neuropsychological measures in amyotrophic lateral sclerosis and their relationship with CT scan-assessed cerebral atrophy
32. Neuropsychological, electroencephalogram and brain computed tomography findings in motor neuron disease
33. 123Iamphetamine single photon emission computer tomography in motor neuron disease
34. 123I-IMP in patients with degenerative dementia
35. 99mTc]-d,l-HMPAO SPECT study
36. Inter-relation between "classic" motor neuron disease and frontotemporal dementia: Neuropsychological and single photon emission computed tomography study
37. Cerebral blood flow and oxygen metabolism in progressive dementia associated with amyotrophic lateral sclerosis
38. Frontal lobe function in amyotrophic lateral sclerosis: A neuropsychological and positron emission tomography study
39. A positron emission tomography study of frontal lobe function (verbal fluency) in amyotrophic lateral sclerosis
40. Cognitive deficits in nondemented amyotrophic lateral sclerosis patients: A neuropsychological investigation
41. Presenile dementia with motor neuron disease in Japan: Clinico-pathological review of 26 cases
42. New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis
43. Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia
44. Involvement of the amygdala, dentate and hippocampus in motor neuron disease
45. Cognitive impairment in sporadic ALS. A pathological continuum underlying a multisystem disorder
46. The pathology of Pick complex
47. The new neuropathology of degenerative frontotemporal dementias
48. Dementia lacking distinctive histopathology: Clinicopathological evaluation of 32 cases
49. Total manifestations of amyotrophic lateral sclerosis
50. Development of ophthalmoplegia in amyotrophic lateral sclerosis during longterm use of respirators
51. Amyotrophic lateral sclerosis patients living beyond respiratory failure
52. A long surviving case of amyotrophic lateral sclerosis with atrophy of the frontal lobe: A comparison with the Mitsuyama type
53. Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22
54. Clinical characteristics of a family with chromosome 17-linked disinhibition-dementiaparkinsonism-amyotrophy complex
55. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
56. ALS, SOD and peroxynitrite
57. Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant
58. Elevated "hydroxyl radical" generation in vivo in an animal model of amyotrophic lateral sclerosis
59. Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis
60. Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis
61. Oxidative stress, mutant SOD1, and neurofilament pathology in transgenic mouse models of human motor neuron disease
62. Familial amyotrophic lateral sclerosis with a mutation in exon 4 of the Cu/Zn superoxide dismutase gene: Pathological and immunocytochemical changes
63. Familial amyotrophic lateral sclerosis with a point mutation of SOD-1: Intrafamilial heterogeneity of disease duration associated with neurofibrillary tangles
64. SOD1 mutation is associated with accumulation of neurofilaments in amyotrophic lateral sclerosis
65. Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement
66. Familial amyotrophic lateral sclerosis with a mutation in the Cu/Zn superoxide dismutase gene
67. Amyotrophic lateral sclerosis associated with homozygosity for a Asp90Ala mutation in CuZn-superoxide dismutase
68. Autosomal recessive adult-onset amyotrophic lateral sclerosis associated with homozygosity for Asp90Ala CuZn-superoxide dismutase mutation. A clinical and genealogical study of 36 patients
69. Motor neuron degeneration in mice that express a human CuZn superoxide dismutase mutation
70. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions
71. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions
72. Copper/Zinc superoxide dismutase mRNA levels are increased in sporadic amyotrophic lateral sclerosis
73. Amyotrophic lateral sclerosis and structural defects in CuZn superoxide dismutase
74. Down-regulation of copper/zinc superoxide dismutase causes apoptotic death in PC12 neuronal cells
75. Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotitc gene: Studies in yeast and neural cells
76. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
77. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
78. Do posttranslational modifications of CuZnSOD lead to sporadic amyotrophic lateral sclerosis?
79. Caspase-1 and -3 are sequentially activated in motor neuron death in CuZn superoxide dismutase-mediated familial amyotrophic lateral sclerosis
80. Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model
81. c-Jun, JNK/SAPK kinases and transcription fact NF-kB are selectively activated in astrocytes, but not motor neurons, in amyotrophic lateral sclerosis
82. Motor neuronal death in amyotrophic lateral sclerosis (ALS) is not apoptotic. A comparative analysis of ALS and chronic aluminum neurotoxicity in New Zealand white rabbits
83. Alterations in skin glycosaminoglycans in patients with ALS
84. Cytopathology of amyotrophic lateral sclerosis
85. Familial amyotrophic lateral sclerosis
86. Motor neuron inclusions in ALS are heavily ubiquitinated
87. Immunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis
88. Cytoskeletal pathology in motor neuron disease
89. Neuropathology of amyotrophic lateral sclerosis: New perspectives on an old disease
90. Characterization of neuronal intermediate filament protein expression in cervical spinal motor neurons in sporadic amyotrophic lateral sclerosis (ALS)
91. Peripherin immunoreactive structures in amyotrophic lateral sclerosis
92. Molecular factors underlying selective vulnerability of motor neurons to neurodegeneration in amyotrophic lateral sclerosis
93. Neurofilament architecture combines structural principles of intermediate filaments with carboxy-terminal extensions increasing in size between triplet proteins
94. Assembly of type IV neuronal intermediate filaments in nonneuronal cells in the absence of preexisting cytoplasmic intermediate filaments
95. Neurofilaments are obligate heteropolymers in vivo
96. Identification of Ser-55 as a major protein kinase A phosphorylation site on the 70-kDa subunit of neurofilaments
97. Dynamics of neuronal intermediate filaments: A developmental perspective
98. Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosis
99. Mice overexpressing the human neurofilament heavy gene as a model of ALS
100. Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
101. Late-onset death of motor neurons in mice overexpressing wild-type peripherin
102. Neurofilament light and polyadenylated mRNA levels are decreased in amyotrophic lateral sclerosis motor neurons
103. Superoxide dismutase catalyzes nitration of tyrosines by peroxynitrite in the rod and head domains of neurofilament-L
104. Nitric oxide synthase expression in cervical motor neurons of sporadic amyotrophic lateral sclerosis
105. Role of SOD-1 and nitric oxide/cyclic GMP cascade on neurofilament aggregation in ALS/MND
106. Copper/zinc superoxide dismutase expression in the human nervous system. Correlation with selective neuronal vulnerability
107. Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons
108. Immunocytochemical study of the distribution of the free radical scavenging enzymes Cu/Zn superoxide dismutase (SOD1); MN superoxide dismutase (MN SOD) and catalase in the normal human spinal cord and in motor neurons
109. Colocalization of NOS and SOD1 in neurofilament accumulation within motor neurons of amyotrophic lateral sclerosis: An immunohistochemical study
110. Increased 3-nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis
111. Increased 3-nitrotyrosine and oxidative damage in mice with a human copper/zinc superoxide dismutase mutation
112. Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhance catalysis of tyrosine nitration by peroxynitrite
113. Nitration of the low molecular weight neurofilament (NFL) is equivalent in sporadic amyotrophic lateral sclerosis and control cervical spinal cord
114. In vitro reactive nitrating species toxicity in dissociated spinal motor neurons from NFL (-/-) and HNFL transgenic mice
115. Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis
116. Analysis of the KSP repeat of the neurofilament heavy subunit in familial amyotrophic lateral sclerosis
117. Novel insertion in the KSP region of the neurofilament heavy gene in amyotrophic lateral sclerosis (ALS)
118. Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis
119. Sequence variants in human neurofilament proteins: Absence of linkage to familial amyotrophic lateral sclerosis
120. Phosphorylation state of the native high molecular weight neurofilament subunit protein (NFH) from cervical spinal cord in sporadic amyotrophic lateral sclerosis
121. Ultrastructural evidence for altered calcium in motor nerve terminals in amyotrophic lateral sclerosis
122. Functional and morphometric study of the liver in motor neuron disease
123. Hepatic ultrastrucutral changes and liver dysfunction in amyotrophic lateral sclerosis
124. Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis
125. Decreased cytochrome C oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis
126. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis
127. Metabolic dysfunction in familial, but not sporadic, amyotrophic lateral sclerosis
128. Mitochondria in sporadic amyotrophic lateral sclerosis
129. Amyotrophic lateral sclerosis: Oxidative enery metabolism and calcium homeostasis in peripheral blood lymphocytes
130. Parvalbumin and calbindin D-28K in the human motor system and in motor neuron disease
131. The role of calciumbinding proteins in selective motoneuron vulnerability in amyotrophic lateral sclerosis
132. Parvalbumin is a marker of ALS-resistant motor neurons
133. Intracellular calcium parallels motoneuron degeneration in SOD-1 mutant mice
134. Calcineurin A and calbindin immunoreactivity in the spinal cord of G93A superoxide dismutase transgenic mice
135. Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motor neuron disease
136. Blood superoxide dismutase, catalase and glutathione peroxidase activities in familial and sporadic amyotrophic lateral sclerosis
137. Brain superoxide dismutase, catalase, and glutathione peroxidase activities in amyotrophic lateral sclerosis
138. Oxidative damage to protein in sporadic motor neuron disease spinal cord
139. Apparent hydroxyl radical production by peroxynitrite: Implications for endothelial injury from nitric oxide and superoxide
140. Chronic inhibition of glutamate uptake produces a model of slow neurotoxicity
141. Motor neurons are selectively vulnerable to AMP/Kianate receptor-mediated injury in vitro
142. Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis
143. Aberrant RNA processing in a neurodegenerative disease: The cause for absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis
144. Polymorphisms in the glutamate transporter gene EAAT2 in European ALS patients
145. Studies of the coding region of the neuronal glutamate transporter gene in amyotrophic lateral sclerosis
146. The EAAT2 (GLT-1) gene in motor neuron disease: Absence of mutations in amyotrophic lateral sclerosis and a point mutation in patients with hereditary spastic paraplegia
147. Glutamatergic receptors regulate expression, phosphorylation and accumulation of neurofilaments in spinal cord neurons
148. Strong expression of class II major histocompatibility complex (MHC) antigens in the absence of detectable T cell infiltration in amyotrophic lateral sclerosis (ALS) spinal cord
149. Major histocompatibility complex antigen expression in the affected tissues in amyotrophic lateral sclerosis
150. Lymphocyte infiltration in the spinal cord of patients with amyotrophic lateral sclerosis
151. Immunological reactions in amyotrophic lateral sclerosis brain and spinal cord tissue
152. Perineuronal glial response after axotomy of central and peripheral axons. A comparison
153. Microglial-neuronal interactions
154. Treatment of the adult retina with microglia-suppressing factors retards axotomy-induced neuronal degradation and enhances axonal regeneration in vivo and in vitro
155. The relationship of microglial cells to dying neurons during natural neuronal cell death and axotomy-induced degeneration of the rat retina
156. Inhibition of mononuclear phagocytes reduces ischemic injury in the spinal cord
157. An investigation into the early stages of the inflammatory response following ibotenic acid-induced neuronal degeneration
158. Murine brain macrophages induce NMDA receptor mediated neurotoxicity in vitro by secreting glutamate
159. Elevation of the neurotoxin quinolinic acid occurs following spinal cord trauma
160. Cytotoxic effect of brain macrophages on developing neurons
161. Reactive microglia in cerebral ischemia: An early mediator of tissue damage?
162. Microglial cells prevent nitric oxide-induced neuronal apoptosis in vitro
163. 3 in New Zealand white rabbits
164. A morphological analysis of the motor neuron degeneration and microglial reaction in acute and chronic in vivo aluminum chloride neurotoxicity
165. Amyotrophic lateral sclerosis: Interleukin-6 levels in cerebrospinal fluid
166. Cerebrospinal fluid interleukin-6 in amyotrophic lateral sclerosis: Immunological parameter and comparison with inflammatory and noninflammatory central nervous system diseases
167. Amyotrophic lateral sclerosis
168. Dose-ranging study of riluzole in amyotrophic lateral sclerosis
169. An analysis of extended survival in patients with amyotrophic lateral sclerosis treated with riluzole
170. Recovery of Nacetylaspartate in corticomotor neurons in patients with ALS after riluzole therapy
171. A double-blind, placebo-controlled trial of L-threonine in amyotrophic lateral sclerosis
172. A controlled trial of amino acid therapy in amyotrophic lateral sclerosis: I. Clinical, functional, and maximum isometric torque data
173. Branched-chain amino acids and amyotrophic lateral sclerosis: A treatment failure?
174. A clinical trial of dextromethorphan in amyotrophic lateral sclerosis
175. Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis
176. Antiglutamate therapy in amyotrophic lateral sclerosis: A trial using lamotrigine
177. A clinical trial of verapamil in amyotrophic lateral sclerosis
178. Effect of recombinant human insulin-like growth factor on progression of ALS. A placebo controlled study
179. Cost effectiveness of recombinant human insulin-like growth factor I therapy in patients with ALS
180. A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis
181. A double-blind, placebo-controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis
182. A placebo-controlled trial of recombinant human ciliary neurotrophic (rhCNTF) factor in amyotrophic lateral sclerosis
183. Recombinant growth hormone treatment of amyotrophic lateral sclerosis
184. Controlled trial of thyrotropin releasing hormone in amyotrophic lateral sclerosis
185. Amyotrophic lateral sclerosis: Effects of acute intravenous and chronic subcutaneous administration of thyrotropin-releasing hormone in controlled trials
186. Amyotrophic lateral sclerosis: A double-blind crossover trial of thyrotropinreleasing hormone
187. A double-blind, placebo-controlled trial of TRH in amyotrophic lateral sclerosis
188. Failure of immunosuppression with a ten- to 14-day course of high-dose intravenous cyclophosphamide to alter the progression of amyotrophic lateral sclerosis
189. Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis
190. Immunosuppressive treatment of motor neuron syndromes
191. Intravenous immunoglobulin therapy in amyotrophic lateral sclerosis
192. Plasmapheresis in amyotrophic lateral sclerosis
193. Plasmapheresis with immunosuppression in amyotrophic lateral sclerosis
194. Trial of immunosuppression in amyotrophic lateral sclerosis using total lymphoid irradiation
195. A double-blind study of the effectiveness of cyclosporine in amyotrophic lateral sclerosis
196. Selegiline is ineffective in a collaborative double-blind, placebo-controlled trial for treatment of amyotrophic lateral sclerosis
197. Double blind cross over trial with deprenyl in amyotrophic lateral sclerosis
198. An open-randomized clinical trial of selegeline in amyotrophic lateral sclerosis
199. Amyotrophic lateral sclerosis. A trial of central monoamine metabolism and therapeutic trial of levodopa
200. Trial of oral physostigmine in amyotrophic lateral sclerosis
201. Cholinesterase inhibitors lack therapeutic effect in amyotrophic lateral sclerosis. A controlled study of physostigmine versus neostigmine
202. A double-blind placebo-controlled study of 3,4-diaminopyridine in amyotrophic lateral sclerosis patients on a rehabilitation unit
203. Functional and pharmacokinetic studies of tetrahydroaminoacridine in patients with amyotrophic lateral sclerosis
204. Therapeutic trial of tilorone in ALS: Lack of benefit in a double-blind, placebo-controlled study
205. Modified snake venom in amyotrophic lateral sclerosis
206. Genetic disorders of motor neurons
207. Linkage of a gene causing familial amyotrophic lateral sclerosis to chromosome 21 and evidence of genetic-locus heterogeneity
208. Linkage of the gene for an autosomal dominant form of juvenile amyotrophic lateral sclerosis to chromosome 9q34
209. Autosomal dominant juvenile amyotrophic lateral sclerosis
210. Juvenile familial amyotrophic lateral sclerosis: Four cases with long survival
211. Linkage of recessive familial amyotrophic lateral sclerosis to chromosome 2q33-q35
212. Linkage of a common locus for recessive amyotrophic lateral sclerosis
213. Paralysie bulbo-pontine chronique progressive familiale avec surdité. Un cas de syndrome de Klippel-Trenaunay dans la même fratrie. Problèmes diagnostiques et génétiques
214. Progressive proximal spinal and bulbar muscular atrophy of late onset
215. X-linked recessive bulbospinal neuronopathy: A report of ten cases
216. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
217. Spinobulbar muscular atrophy can mimic ALS: The importance of genetic testing in male patients with atypical ALS
218. Motor neuron disease and adult hexosaminidase A deficiency in two families: Evidence for multisystem degeneration
219. N-acetyl-b-hexosaminidase b locus defect and juvenile motor neuron disease: A case study
220. Adult onset motor neuronopathy in the juvenile type of hexosaminidase A and B deficiency
221. Molecular basis of an adult form of b-hexosaminidase B deficiency with motor neuron disease
222. Genetics of amyotrophic lateral sclerosis: An overview