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https://www.readbyqxmd.com/read/25957009/-normal-pressure-hydrocephalus
#1
REVIEW
J M Lieb, C Stippich, F J Ahlhelm
CLINICAL ISSUE: Normal pressure hydrocephalus (NPH) is a disorder found mainly in the elderly (> 60 years) with an increasing prevalence with age and is one of the few treatable causes of dementia. If untreated NPH often leads to severe motor, psychomotor and irreversible cognitive deficits. The pathogenesis is not yet fully understood. Clinical symptoms consist of the (not always complete) classical triad of equilibrium and gait disturbances followed later by incontinence and dementia...
May 2015: Der Radiologe
https://www.readbyqxmd.com/read/24092290/myoclonus
#2
REVIEW
Alberto J Espay, Robert Chen
PURPOSE OF REVIEW: Myoclonus remains a challenging movement phenotype to characterize, evaluate, and treat. A systematic assessment of the temporal sequence, phenomenology, and distribution of movements can assist in the rational approach to diagnosis and management. RECENT FINDINGS: Cortical forms of myoclonus are increasingly recognized as primarily cerebellar disorders. A syndrome of orthostatic myoclonus has been recognized by electrophysiology in patients with neurodegenerative disorders, mainly in Alzheimer disease, accounting for impairments in gait and balance previously mischaracterized as normal pressure hydrocephalus or orthostatic tremor...
October 2013: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/23904623/antisense-reduction-of-tau-in-adult-mice-protects-against-seizures
#3
Sarah L DeVos, Dustin K Goncharoff, Guo Chen, Carey S Kebodeaux, Kaoru Yamada, Floy R Stewart, Dorothy R Schuler, Susan E Maloney, David F Wozniak, Frank Rigo, C Frank Bennett, John R Cirrito, David M Holtzman, Timothy M Miller
Tau, a microtubule-associated protein, is implicated in the pathogenesis of Alzheimer's Disease (AD) in regard to both neurofibrillary tangle formation and neuronal network hyperexcitability. The genetic ablation of tau substantially reduces hyperexcitability in AD mouse lines, induced seizure models, and genetic in vivo models of epilepsy. These data demonstrate that tau is an important regulator of network excitability. However, developmental compensation in the genetic tau knock-out line may account for the protective effect against seizures...
July 31, 2013: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/23250022/-diagnosis-of-normal-pressure-hydrocephalus-in-elderly-patients-a-review
#4
REVIEW
Marc Verny, Gilles Berrut
The definition of normal pressure hydrocephalus (NPH), in adults, associates clinical signs (Adams and Hakim triad) involving gait disorders, urinary incontinence and dementia, associated with aspects on brain imaging that are consistent with this hypothesis and also normal or slightly increased intracranial pressure. The aim of this study was to clarify the techniques and methods facilitating the diagnosis of NPH. The literature review has been conducted from the Medline database without date limitation including the keywords "normal pressure hydrocephalus" and "diagnosis...
December 2012: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
https://www.readbyqxmd.com/read/22764244/mir-886-3p-levels-are-elevated-in-friedreich-ataxia
#5
Lata H Mahishi, Ronald P Hart, David R Lynch, Rajiv R Ratan
Friedreich ataxia (FRDA) is the most common inherited ataxia caused primarily by an intronic GAA.TTC triplet repeat expansion in the frataxin (FXN) gene. FXN RNA and protein levels are reduced in patients leading to progressive gait and limb ataxia, sensory loss, reduced tendon reflexes, dysarthria, absent lower limb reflexes, and loss of position and vibration sense. Neurological manifestations ensue from primary loss of dorsal root ganglia neurons and their associated axons ascending centrally in the spinal cord and peripherally in large myelinated nerves...
July 4, 2012: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/21134000/an-autopsy-case-of-an-aged-patient-with-spinocerebellar-ataxia-type-2
#6
REVIEW
Chiho Ishida, Kiyonobu Komai, Kohei Yonezawa, Ken-Ichi Sakajiri, Eishun Nitta, Atsuhiro Kawashima, Masahito Yamada
We report the case of a woman who developed limb clumsiness in her fifties and gait disturbance in her sixties. She was bedridden after bone fractures at age 75 and showed disorientation, slow eye movement, gaze palsy, ataxic speech, muscle atrophy and weakness, and areflexia with pathological reflex. She died of respiratory failure at age 85. This patient was diagnosed genetically as having spinocerebellar ataxia type 2 (SCA2), and the number of expanded CAG repeats was 41. At autopsy, the brain weighed 965 g, and the brainstem, cerebellum, frontal convexity and spinal cord were atrophic...
October 2011: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/18312021/when-to-consider-normal-pressure-hydrocephalus-in-the-patient-with-gait-disturbance
#7
REVIEW
Ronan Factora, Mark Luciano
Normal pressure hydrocephalus (NPH) is a chronic adult disorder of unknown cause. It is characterized by gradual onset of gait impairment, cognitive dysfunction, and urinary incontinence in the presence of enlarged ventricles. NPH is a relatively rare cause of these 3 common symptoms. Diagnosis is made based on suspicion of NPH symptoms, the additional finding of ventriculomegaly on imaging, and confirmatory testing with a trial of CSF drainage, which can predict improvement with CSF shunting. The differential diagnosis must consider common causes of each of the symptoms and include Alzheimer's disease (AD), Parkinson's disease (PD), vascular dementia, and spinal stenosis...
February 2008: Geriatrics
https://www.readbyqxmd.com/read/15960171/-a-case-of-primary-progressive-multiple-sclerosis-with-onset-of-memory-impairment
#8
Ken-ichiro Yamashita, Takuo Nomura, Yasumasa Ohyagi, Takayuki Taniwaki, Hirokazu Furuya, Yasuo Kuwabara, Jun-ichi Kira
We report a 52-year-old woman with primary progressive multiple sclerosis (PPMS) presenting with chronic progressive memory impairment. From a couple of years prior to admission, she had developed impairment of her short-term memory. For example, she forgot her nephew's name, and spoke the same phrases again and again. She also sometimes forgot to turn off her gas stove and forgot things she bought in shops. Moreover, her mental activity gradually decreased and she became apathetic. However, she did not note her memory impairment, and had no hallucinations...
May 2005: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/10466866/cervical-spondylarthrotic-myelopathy-with-early-onset-in-down-s-syndrome-five-cases-and-a-review-of-the-literature
#9
REVIEW
G P Bosma, M A van Buchem, J H Voormolen, F C van Biezen, O F Brouwer
Progressive walking difficulties and bladder dysfunction may be attributed to Alzheimer disease or atlanto-axial subluxation in people with Down's syndrome (DS). The present authors describe five patients with DS suffering from the above symptoms as a result of cervical spondylarthrotic myelopathy. Clinical and radiological data were collected from all patients with DS who underwent surgery for cervical spondylarthrotic myelopathy at the Leiden University Medical Centre during the period between 1991 and 1995...
August 1999: Journal of Intellectual Disability Research: JIDR
https://www.readbyqxmd.com/read/9282374/-a-76-year-old-man-with-loss-of-vision-and-dementia
#10
N Miyashita, H Imai, H Mori, M Kodera, T Shirai, Y Mizuno
We report a 76-year-old man who developed blurred vision and dementia. He was apparently well until April 4, 1990 (70-year-old at that time) when he had a sudden onset of bilateral loss of vision. Corrected vision was 0.1 (right) and 0.09 (left). He was admitted to the ophthalmology service of our hospital on April 9, 1990, and neurological consultation was asked on April 11. Neurologic examination revealed alert and oriented man without dementia. Higher cerebral functions were intact. He had bilateral large visual field defects with loss of vision; he was only able to count the digit number with his right eye and to recognize hand movement with his left eye...
August 1997: Nō to Shinkei, Brain and Nerve
https://www.readbyqxmd.com/read/8921532/-an-autopsy-case-of-amyotrophic-lateral-sclerosis-with-concomitant-alzheimer-s-and-incidental-lewy-body-diseases
#11
M Su, K Wakabayashi, Y Tanno, T Inuzuka, H Takahashi
We report a 72-year-old man with sporadic amyotrophic lateral sclerosis (ALS) who showed concomitant histopathology of Alzheimer's disease (AD) and incidental Lewy body disease. The patient presented at the age of 70 years with distal upper limb amyotrophy. Thereafter, gait disturbance and respiratory distress progressed. Neuropathological examination showed mild frontal lobe and anterior spinal root atrophy. There was moderate loss of upper and lower motor neurons, and Bunina bodies and skein-like inclusions were present in the spinal anterior horns and facial and hypoglossal nuclei, confirming the pathology of ALS...
October 1996: Nō to Shinkei, Brain and Nerve
https://www.readbyqxmd.com/read/2625028/-an-autopsy-case-of-multiple-system-atrophy-with-many-senile-plaques
#12
T Ohshima, T Endo, M Kobayashi, S Ikeda, T Onaya
A 60 year-old man was admitted to our hospital because of gait disturbance and dizziness. At 57 years of age, he noticed his walking unstable. After then, he had dizziness due to orthostatic hypotension, urinary difficulty, loss of livid, and forgetfulness. Neurological examination revealed he had severe orthostatic hypotension, cerebellar ataxia, dysarthria, hyperreflexia of four limbs, myoclonus of right leg, and atonic bladder. His brain CT showed cerebellar atrophy. Thereafter he had recurrent syncopic attacks...
November 1989: Rinshō Shinkeigaku, Clinical Neurology
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