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Dementia and muscle wasting

Régis Gonthier
Falls are the leading cause of accidental death among the elderly, accounting for about 12 000 deaths per year. The risk of falls increases with age: one-third of subjects over 65 years old living in the community and half of those over 85 fall at least once a year. Despite their frequency, falls should not be trivialized, as they are associated with significant morbidity and care expenditure: 10% of falls have severe traumatic consequences, and30 % of hospital admissions for traumatic injury among elderly individuals are due to falls...
June 2014: Bulletin de L'Académie Nationale de Médecine
Norihiko Terada, Kensuke Kinoshita, Shijima Taguchi, Yasuharu Tokuda
Deficiency of multiple vitamins can be identified in alcoholic and malnourished patients. We report a patient with Wernicke encephalopathy, a B1 deficiency and pellagra, a niacin deficiency. A 61-year-old Japanese man presented with generalised weakness. He had drunk alcohol heavily for more than a year without eating adequate meals. Physical examination showed disorientation, eye movement impairment, muscle wasting and a rash over the limbs. Multivitamin supplementations improved all the symptoms. Pellagra causes dementia, diarrhoea, or dermatitis, and can mimic non-specific erythaema in alcoholics...
2015: BMJ Case Reports
L St John, A J Larner
No abstract text is available yet for this article.
October 2015: British Journal of Hospital Medicine
Robin M Daly, Jenny Gianoudis, Melissa Prosser, Dawson Kidgell, Kathryn A Ellis, Stella O'Connell, Caryl A Nowson
BACKGROUND: Age-related muscle wasting has been strongly implicated with falls and fractures in the elderly, but it has also been associated with cognitive decline and dementia. Progressive resistance training (PRT) and adequate dietary protein are recognised as important contributors to the maintenance of muscle health and function in older adults. However, both factors also have the potential to improve brain function and prevent cognitive decline via several pathways, including the regulation of various growth and neurotrophic factors [insulin-like growth factor-1 (IGF-1)]; brain-derived growth factor (BDNF)] and/or the modulation of systemic inflammation...
2015: Trials
Sabine Krause
Muscle mass and function are gradually lost in age-related, degenerative neuromuscular disorders, which also reflect the clinical hallmarks of sarcopenia. The consensus definition of sarcopenia includes a condition of age-related loss of muscle mass, quality, and strength. The most common acquired muscle disease affecting adults aged over 50 years is sporadic inclusion body myositis (sIBM). Besides inflammatory effects and immune-mediated muscle injury, degenerative myofiber changes are characteristic features of the disease...
2015: Frontiers in Aging Neuroscience
Eric Dec, Prachi Rana, Veeral Katheria, Rachel Dec, Manaswitha Khare, Angèle Nalbandian, Szu-Yun Leu, Shlomit Radom-Aizik, Katrina Llewellyn, Lbachir BenMohamed, Frank Zaldivar, Virginia Kimonis
Valosin containing protein (VCP) disease (also known as Inclusion Body Myopathy, Paget Disease of Bone and Frontotemporal Dementia [IBMPFD] syndrome) is caused by mutations in the gene encoding VCP classically affecting the muscle, bone and brain. Although the genetic cause has been identified, details regarding the pathogenesis of IBMPFD have not been fully determined. Muscle wasting observed in VCP disease is suggestive of cytokine imbalance. We hypothesized that dysfunctional protein homeostasis caused by VCP mutations leads to cytokine imbalances thereby contributing to the muscle wasting phenotype...
February 2014: Clinical and Translational Science
S Spina, A D Van Laar, J R Murrell, R L Hamilton, J K Kofler, F Epperson, M R Farlow, O L Lopez, J Quinlan, S T DeKosky, B Ghetti
BACKGROUND AND PURPOSE: The phenotype of IBMPFD [inclusion body myopathy with Paget's disease of the bone and frontotemporal dementia (FTD)] associated with valosin-containing protein (VCP) mutation is described in three families. METHODS: Probands were identified based on a pathological diagnosis of frontotemporal lobar degeneration with TDP-43-positive inclusions type IV. VCP sequencing was carried out. Clinical data on affected family members were reviewed. RESULTS: Ohio family: four subjects presented muscle weakness and wasting...
February 2013: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Z Shi, Y K Hayashi, S Mitsuhashi, K Goto, D Kaneda, Y-C Choi, C Toyoda, S Hieda, T Kamiyama, H Sato, M Wada, S Noguchi, I Nonaka, I Nishino
BACKGROUND AND PURPOSE: Mutations in the valosin-containing protein (VCP) gene are known to cause inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) and familial amyotrophic lateral sclerosis (ALS). Despite an increasing number of clinical reports, only one Asian family with IBMPFD has been described. METHODS: To characterize patients with VCP mutations, we screened a total of 152 unrelated Asian families who were suspected to have rimmed vacuolar myopathy...
March 2012: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Katsuya Nakamura, Kunihiro Yoshida, Tsuneaki Yoshinaga, Minori Kodaira, Yasuhiro Shimojima, Yo-Ichi Takei, Hiroshi Morita, Katsuhiko Kayanuma, Shu-Ichi Ikeda
Triple A syndrome is caused by mutations in the gene encoding ALADIN, leading to achalasia, alacrima and addisonism. Neurologic manifestations of the disease include motor neuron disease-like presentations, motor-sensory or autonomic neuropathy, optic atrophy, cerebellar ataxia, Parkinsonism, and mild dementia. We report a 60-year-old Japanese man with triple A syndrome. He was born to non-consanguineous parents. He underwent a surgical operation for achalasia at age 40, and thereafter, he developed a slowly progressive gait disturbance...
October 15, 2010: Journal of the Neurological Sciences
Nobuyoshi Fukuhara
MERRF is an acronym of myoclonus epilepsy associated with ragged-red fibers and was first reported as a new nosological entity belonging to mitochondrial encephalomyopathies in San Remo symposium on "Mitochondrial Pathology" in 1982. MERRF was named Fukuhara disease by Rowland (1983). The first reported patient had been diagnosed as having Ramsay Hunt syndrome associated with Friedreich's ataxia. However, nowadays, the previously reported cases as having Ramsay Hunt syndrome associated with Friedreich's ataxia are regarded as having been suffered from MERRF...
January 2008: Brain and Nerve, Shinkei Kenkyū No Shinpo
Per M Roos, Olof Vesterberg, Monica Nordberg
Degenerative processes within the nervous system are common features in disease entities such as dementia of Alzheimer type (DAT), Parkinson disease (PD), and amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative disease with unknown etiology; widespread muscle wasting and respiratory failure lead to death within a few years. Denervation can be detected with electromyography and axonal deterioration monitored by motor unit number estimates. Several suggestions about the cause of ALS have emerged but no solid theory has yet precipitated...
October 2006: Experimental Biology and Medicine
Yasuko Toyoshima, Chun-Feng Tan, Tetsuro Kozakai, Masaharu Tanaka, Hitoshi Takahashi
We report the autopsy findings of a 62-year-old man who exhibited progressive FTD 10 years before the appearance of muscle weakness and wasting, and who died approximately 11 years after onset of the symptoms. Degeneration and atrophy of the frontal and temporal lobes, which contained ubiquitin-positive neuronal inclusions and dystrophic neurites, were evident. Circumscribed degeneration affecting the hippocampal CA1-subiculum border zone was also a feature. Moreover, degeneration was present in both the upper and lower motor neuron systems, the latter being more severely affected...
September 2005: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Harold D Foster
HIV-1 encodes for one of the human glutathione peroxidases. As a consequence, as it is replicated, its genetic needs cause it to deprive HIV-1 seropositive individuals not only of glutathione peroxidase, but also of the four basic components of this selenoenzyme, namely selenium, cysteine, glutamine, and tryptophan. Eventually this depletion process causes severe deficiencies of all these substances. These, in turn, are responsible for the major symptoms of AIDS which include immune system collapse, greater susceptibility to cancer and myocardial infarction, muscle wasting, depression, diarrhea, psychosis and dementia...
2004: Medical Hypotheses
Bungo Okuda, Yasumichi Iwamoto, Nobuyuki Oka, Hisao Tachibana
We report a 61-year-old, right-handed woman with motor neuron disease, dementia, and apraxia of the upper limbs. The patient developed clumsiness of the right hand and dysarthria two years and a half prior to admission. Neurological examination showed limb-kinetic apraxia and ideomotor apraxia, predominantly on the right side, in addition to dementia and anarthria. There was mild muscle wasting in the neck and hands. A muscle biopsy from the biceps muscle of arm as well as needle EMG revealed neurogenic changes compatible with motor neuron disease...
October 2002: Rinshō Shinkeigaku, Clinical Neurology
Atsushi Ishikawa, Mitsunori Yamada, Kunihiko Makino, Izumi Aida, Jiro Idezuka, Takeshi Ikeuchi, Yoshiaki Soma, Hitoshi Takahashi, Shoji Tsuji
BACKGROUND: Machado-Joseph disease (MJD; spinocerebellar ataxia type 3) is a hereditary neurodegenerative disease caused by mutation of the MJD1 gene. Patients with MJD usually present with cerebellar ataxia, external ophthalmoplegia, pyramidal and extrapyramidal signs, and muscle wasting. However, it has been reported that these patients do not demonstrate dementia. CASE DESCRIPTION: We noticed symptoms of dementia and delirium in 4 patients with MJD. The symptoms included abnormal behavior, excitation, an uncooperative attitude, crying, disorientation, slow thought processes, hallucinations, and delusions...
November 2002: Archives of Neurology
T Kaneto, K Inoue, K Shimoda, M Doi, T Kitagawa, H Kumada, H Miyata, E Ohama
A 42-year-old man without family history of neurologic disease developed muscle weakness, and wasting associated with tremor and choreiform movement. He died at age 75 with 33 years of total duration of illness. Autopsy revealed neuronal loss and gliosis in the anterior horns of the spinal cord, hypoglossal nuclei, caudate nuclei, putamen, globus pallidus, and substantia nigra. Bunina bodies or Lewy bodies were not seen. Several similar cases with motor neuron disease, dementia and parkinsonism have been reported in the literature...
August 2000: Rinshō Shinkeigaku, Clinical Neurology
E Nitta, A Ishikawa, H Ishiguro, H Baba, N Fukuhara
A case of familial juvenile parkinsonism with dementia, orthostatic hypotension, neurogenic bladder and constipation was reported. He had been in a good health until the age of 28 when a finger tremor occurred on effort to hold hands in a definite position, and disturbances in gait and speech were noted. These symptoms were relieved by levodopa treatment followed by dyskinesia and motor fluctuations. Three years later, he complained of faintness, constipation and urinary frequency. The neurological examination revealed mentally sound male with masked face, tremor and rigidity in his extremities, and short step gait with lateropulsion...
January 1993: Rinshō Shinkeigaku, Clinical Neurology
K M Chapman, R A Nelson
Loss of appetite, regardless of cause, is a significant symptom that may cause extreme weight loss in older patients. The consequences of anorexia include muscle wasting, weakness, depression, increased susceptibility to disease complications, and decreased immunocompetence. In the older population, the major causes of anorexia are pulmonary and cardiac diseases, cancer, dementia, alcoholism, depression, and medications. The diagnosis is based on the physical exam, a history of weight loss, and a review of dietary intake...
March 1994: Geriatrics
T Nomura, M Ota, N Kotake, K Tanaka
A 73-year-old woman (patient 1) developed progressive mental deterioration at age 63, and seizures at age 70. On examination, she showed severe dementia, tonic clonic convulsion, hypotonia and muscular wasting. There was neither myoclonus nor cerebellar ataxia. Brain CT revealed a low density area in the right occipital lobe. A 44-year-old man (son of the patient 1) developed unsteady gait at age 15, muscle twitching at age 18 and then noticed speech disturbance at age 35. He had no history of convulsive seizure...
November 1993: Rinshō Shinkeigaku, Clinical Neurology
T Lynch, M Sano, K S Marder, K L Bell, N L Foster, R F Defendini, A A Sima, C Keohane, T G Nygaard, S Fahn
We studied the clinical features, pathology, and molecular genetics of a family (Mo) with an autosomal dominant disinhibition, frontal lobe dementia, parkinsonism, and amyotrophy. We examined seven affected members and gathered clinical information on another six. The mean onset was at age 45 years. Personality and behavioral changes (disinhibition, withdrawal, alcoholism, hyperphagia) were the first symptoms in twelve. There was early memory loss, anomia, and poor construction with preservation until late of orientation, speech, and calculations...
October 1994: Neurology
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