scholarly journals An Unusual Subacute Progressive Motor Neuronopathy with Myasthenia-like Features

1988 ◽  
Vol 15 (3) ◽  
pp. 304-309 ◽  
Author(s):  
J.H. Noseworthy ◽  
A.D. Rae-Grant ◽  
W.F. Brown
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Unusual Case ◽  
Clinical Course ◽  
Subsequent Development ◽  
Response To Treatment ◽  
Pathological Features ◽  
Pathological Findings ◽  
Unusual Variant ◽  
Lateral Sclerosis ◽  
Clinical And Pathological Features

ABSTRACT:The initial presentation and clinical course of this 60-year old woman suggested a diagnosis of myasthenia gravis. The subsequent development of tongue fasciculations and the lack of response to treatment made a diagnosis of amyotrophic lateral sclerosis (ALS) more likely despite the presence of conjugate gaze paresis and the absence of many of the typical clinical and electromyographic (EMG) findings seen in this condition. The pathological findings were consistent with either a motor neuronopathy or an unusual variant of ALS. We review the clinical and pathological features of this unusual case in this report.

scholarly journals Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p

2012 ◽  
Vol 123 (3) ◽  
pp. 409-417 ◽  
Author(s):  
Heather Stewart ◽  
Nicola J. Rutherford ◽  
Hannah Briemberg ◽  
Charles Krieger ◽  
Neil Cashman ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Pathological Features ◽  
C9orf72 Gene ◽  
Chromosome 9P ◽  
Lateral Sclerosis ◽  
Clinical And Pathological Features

scholarly journals Amyotrophic Lateral Sclerosis/Parkinsonism/Dementia: Clinico-Pathological Correlations Relevant to Guamanian ALS/PD

1991 ◽  
Vol 18 (S3) ◽  
pp. 387-389 ◽  
Author(s):  
Arthur J. Hudson
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Nervous System ◽  
Substantia Nigra ◽  
Globus Pallidus ◽  
Neurofibrillary Tangles ◽  
Senile Plaques ◽  
Lewy Bodies ◽  
Pathological Features ◽  
Lateral Sclerosis ◽  
Clinical And Pathological Features

ABSTRACT:In a recent report on the clinical and pathological features of Guamanian ALS/PD and post-encephalitic parkinsonism/ALS a number of similarities were described, notably in the distribution of neurofibrillary tangles throughout the nervous system. In this account additional pathological features which these disorders share (and which differ from classical ALS, Parkinson's and Alzheimer's diseases) are described. These include atrophy of the globus pallidus and the entire substantia nigra, viz. pars compacta and pars reticulata. Moreover, neither Lewy bodies nor senile plaques are features of the Guamanian and post-encephalitic disorders. The significance of these observations and their relationship, more generally, to parkinsonism, ALS and dementia are discussed.

scholarly journals Motor Unit Number Estimation in Neuromuscular Disease

2008 ◽  
Vol 35 (2) ◽  
pp. 153-159 ◽  
Author(s):  
Omid Rashidipour ◽  
K. Ming Chan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Neuromuscular Disease ◽  
Muscular Atrophy ◽  
Neuromuscular Diseases ◽  
Neuronal Loss ◽  
Response To Treatment ◽  
Motor Unit Number Estimation ◽  
Electrophysiological Method ◽  
Lateral Sclerosis

Motor unit number estimation (MUNE) is an electrophysiological method designed to quantify motor unit loss in target muscles of interest. Most of the techniques are noninvasive and are therefore well suited for longitudinal monitoring. In this brief review, we describe the more commonly used techniques and their applications in amyotrophic lateral sclerosis, poliomyelitis, spinal muscular atrophy and hereditary sensorimotor neuropathies. Findings in some of these studies offer important pathophysiological insights. Since conventional electrophysiologic methods are not sensible measures of motor neuronal loss, MUNE could play a potentially important role in the diagnosis, monitoring of disease progression and response to treatment in neuromuscular diseases in which motor unit loss is a major feature.

A novel SOD1 mutation in a young amyotrophic lateral sclerosis patient with a very slowly progressive clinical course

2010 ◽  
Vol 42 (4) ◽  
pp. 596-597 ◽  
Author(s):  
Eleni Georgoulopoulou ◽  
Cinzia Gellera ◽  
Cinzia Bragato ◽  
Patrizia Sola ◽  
Annalisa Chiari ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Course ◽  
Amyotrophic Lateral Sclerosis Patient ◽  
Sod1 Mutation ◽  
Lateral Sclerosis

CSF chemokine alterations related to the clinical course of amyotrophic lateral sclerosis

2010 ◽  
Vol 222 (1-2) ◽  
pp. 76-81 ◽  
Author(s):  
Takahisa Tateishi ◽  
Ryo Yamasaki ◽  
Masahito Tanaka ◽  
Takuya Matsushita ◽  
Hitoshi Kikuchi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Course ◽  
Lateral Sclerosis

scholarly journals Risk of unsuccessful noninvasive ventilation for acute respiratory failure in heterogeneous neuromuscular diseases: a retrospective study

2017 ◽  
Vol 9 (1) ◽  
Author(s):  
Hiroshi Kataoka ◽  
Hitoki Nanaura ◽  
Kaoru Kinugawa ◽  
Yuto Uchihara ◽  
Hiroya Ohara ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Amyotrophic Lateral Sclerosis ◽  
Respiratory Failure ◽  
Acute Respiratory Failure ◽  
Clinical Course ◽  
Neuromuscular Diseases ◽  
Invasive Ventilation ◽  
Noninvasive Mechanical Ventilation ◽  
To Receive ◽  
Lateral Sclerosis

If invasive ventilation can be avoided by performing noninvasive mechanical ventilation (NIV) in patients with acute respiratory failure (ARF), the disease can be effectively managed. It is important to clarify the characteristics of patients with neuromuscular diseases in whom initial NIV is likely to be unsuccessful. We studied 27 patients in stable neuromuscular condition who initially received NIV to manage fatal ARF to identify differences in factors immediately before the onset of ARF among patients who receive continuous NIV support, patients who are switched from NIV to invasive ventilation, and patients in whom NIV is discontinued. Endpoints were evaluated 24 and 72 hours after the initiation of NIV. After 24 hours, all but 1 patient with amyotrophic lateral sclerosis (ALS) received continuous NIV support. 72 hours later, 5 patients were switched from NIV to invasive ventilation, and 5 patients continued to receive NIV support. 72 hours after the initiation of NIV, the proportion of patients with a diagnosis of ALS differed significantly among the three groups (P=0.039). NIV may be attempted to manage acute fatal respiratory failure associated with neuromuscular diseases, but clinicians should carefully manage the clinical course in patients with ALS.

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