Dementia Accompanying Motor Neuron Disease

López, Oscar L.; Becker, James T.; DeKosky, Steven T.

doi:10.1159/000106693

Cited by 9 publications

(6 citation statements)

References 24 publications

(36 reference statements)

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“…14,[18][19][20] Dementia develops in about 5 percent of patients presenting with sporadic amyotrophic lateral sclerosis and in about 15 percent of patients with the familial form of amyotrophic lateral sclerosis. 14 In both forms the dementia is often very mild, develops late in the course of the disease, and is difficult to evaluate when severe bulbar weakness impairs speech.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…Many cases of dementia with motor neuron disease, with or without extrapyramidal features, have been reported since then. [18][19][20][22][23][24][25][26][27][28][29] The variety of names applied to this clinical syndrome has been confusing. Most authors with an interest in neuromuscular diseases consider it to be a variant of amyotrophic lateral sclerosis, whereas those who approach the disease behaviorally refer to it as the frontotemporal dementia syndrome.…”

Section: Differential Diagnosismentioning

confidence: 99%

See 1 more Smart Citation

Case 12-1999

Drachman¹,

Newell²

1999

N Engl J Med

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A 67-year-old, right-handed man was admitted to the hospital because of increasing dementia.Three years before admission, the patient's cognitive function began to decline. One year later, a neurologist diagnosed progressive dementia with parkinsonism.During the two months before admission, the patient became agitated, and trazodone was prescribed. His family noted transient periods when he stared into space and did not respond to his surroundings. He fell several times, with apparent loss of consciousness but no obvious injury.One week before admission, the patient punched his wife because she would not let him drive. Soon thereafter, he began to look under his bed, locked the doors and windows, and became convinced that the neighbors were trying to steal the family's car. Subsequently, he struck his teenage grandson without provocation and wandered from home. He was referred to this hospital.The patient had boxed professionally for 10 years in over 100 bouts. He drank alcohol excessively in his youth but had been sober for several decades. There was no family history of psychiatric problems. A sibling had died 10 years earlier of probable amyotrophic lateral sclerosis.The temperature was 37°C, the pulse was 84, and the respirations were 16. The blood pressure was 145/80 mm Hg. The results of a general physical examination were normal.On neurologic examination, the patient was alert but was oriented only to himself. He believed that he was in his house in the city and that the year was 1970. He lost concentration when attempting serial tasks. Abstract tasks were performed poorly. He recalled none of three objects at five minutes, even with cues. Remote autobiographical memories were relatively preserved. Left-right confusion was present. The patient's language was intact, and phrases were repeated accurately. His speech was hypophonic. His facial expression was reduced, and he drooled. Cranial-nerve functions were preserved. There was severe atrophy of the shoulder girdles and arms and legs. Diffuse fasciculations and muscle twitches were observed. Motor power was normal. The patient's posture was slightly stooped. His spontaneous gait was normal, and tandem walking was unsteady. No resting tremor or cogwheel rigidity was detected. His sense of joint position and perception of vibration were intact. The deep-tendon reflexes were + or ++ and symmetric. No bradykinesia or postural instability was evident, and the glabellar-tap sign and Babinski signs were absent. The electrocardiographic and laboratory findings were normal.A magnetic resonance imaging (MRI) examination of the brain showed severe, diffuse ventricular and cortical sulcal enlargement (Fig. 1). T 2 -weighted images showed slight thinning of the corpus callosum, atrophy of the mamillary body, a moderately reduced signal in the globus pallidus and putamen, and minimal hyperintensity of periventricular white matter. The pars compacta was thinned on the proton-densityweighted images. A cavum septi pellucidi was noted. The major intracranial vessels were p...

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Section: Differential Diagnosismentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

Case 12-1999

Drachman¹,

Newell²

1999

N Engl J Med

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“…The concept of clinics devoted to the care of ALS patients, pioneered by Stan Appel and Forbes Norris, means that ALS patients, once seen at most a few times by their local neurologist and then cared for by an internist until their death, are now being seen regularly throughout the course of their disease by neurologists who were seeing tens of hundreds of ALS patients rather than one or two per year. As reports of patients with motor neuron disease (MND) associated with dementia were published in increasing numbers [7–18] and the clinical features of frontotemporal dementia were better described including consensus criteria first published in 1998 [19, 20], it became clear that the dementia seen in ALS patients is best characterized as FTD [21, 22]. This review will address what constitutes frontotemporal dysfunction in ALS and how frequently it occurs, how to best evaluate cognition in ALS patients, and what is understood about the pathology and genetics of ALS and FTD.…”

Section: Introductionmentioning

confidence: 99%

ALS and Frontotemporal Dysfunction: A Review

Achi

Rudnicki

2012

Neurology Research International

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Though once believed to be a disease that was limited to the motor system, it is now apparent that amyotrophic lateral sclerosis (ALS) may be associated with cognitive changes in some patients. Changes are consistent with frontotemporal dysfunction, and may range from mild abnormalities only recognized with formal neuropsychological testing, to profound frontotemporal dementia (FTD). Executive function, behavior, and language are the most likely areas to be involved. Screening helpful in detecting abnormalities includes verbal or categorical fluency, behavioral inventories filled out by the caregiver, and evaluation for the presence of depression and pseudobulbar affect. Patients with cognitive dysfunction have shortened survival and may be less compliant with recommendations regarding use of feeding tubes and noninvasive ventilation. Evolving knowledge of genetic and pathological links between ALS and FTD has allowed us to better understand the overlapping spectrum of ALS and FTD.

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“…Further neuropsychological testing with tests sensitive to frontal lobe and subcortical function may be useful in helping to establish a cortical involvement in patients with MND. Lopez et al [4] suggested that slowing of cognitive processes associated with selective motor speed and attentional tasks were more frequent than deficits in planning and conceptualization. As these impaired cognitive processes have been associated with subcortical functioning, they could be of major interest to distinguish D-MND from FLD and Pick's disease before neurological symptoms will be apparent.…”

Section: Discussionmentioning

confidence: 99%

“…Von Poppe and Trennstedt [3] were the first to publish the coincidence of dementia and MND. In subsequent studies, this syndrome was only reported in a few cases [4,5]. In an epidemiological survey, dementia was found in 4% of the MND cases in Japan [6].…”

Section: Introductionmentioning

confidence: 99%