Peripheral neuropathies and motor neuron disorders

Author(s):  
Stéphane Mathis ◽  
Jean-Michel Vallat

Peripheral nervous system disorders are common in older people. Most peripheral neuropathies are secondary to metabolic (diabetes) or toxic causes (medications, alcohol), but several other aetiologies are possible, requiring clinical, electrophysiological, biological, and sometimes pathological (nerve biopsy) examinations to make the right diagnosis and instigate effective treatments. However, despite using this strategy, no cause is found in some axonal polyneuropathies, leading to the concept of chronic idiopathic axonal polyneuropathy (CIAP). Amyotrophic lateral sclerosis (ALS) affects the peripheral nervous system, but is also characterized by central nervous system involvement: it is a strictly motor disorder affecting both the first and second motor neurons, usually presenting during the sixth or seventh decade. This rapidly progressive, still incurable neurodegenerative disease leads to death in all cases.

Author(s):  
Stéphane Mathis ◽  
Pierre Bouche ◽  
Jean-Michel Vallat

Peripheral nervous system disorders are common in older people. Most peripheral neuropathies are secondary to metabolic (diabetes) or toxic causes (medications, alcohol), but several other aetiologies are possible, requiring clinical, electrophysiological, biological, and sometimes pathological (nerve biopsy) examinations to make the right diagnosis and instigate effective treatments. However, despite using this strategy, no cause is found in some axonal polyneuropathies, leading to the concept of chronic idiopathic axonal polyneuropathy (CIAP). Amyotrophic lateral sclerosis (ALS) affects the peripheral nervous system, but is also characterized by central nervous system involvement: it is a strictly motor disorder affecting both the first and second motor neurons, usually presenting during the sixth or seventh decade. This rapidly progressive, still incurable neurodegenerative disease leads to death in all cases.


Author(s):  
Aluisio Rosa Gameiro Filho ◽  
Guilherme Sturzeneker ◽  
Ever Ernesto Caso Rodriguez ◽  
André Maia ◽  
Melina Correia Morales ◽  
...  

Abstract Background To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. Case presentation A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. Conclusions We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.


1979 ◽  
Vol 57 (3) ◽  
pp. 329-332 ◽  
Author(s):  
Peter Ruben ◽  
Ken Lukowiak

We have studied the effects of dopamine on the gill withdrawal reflex evoked by tactile siphon stimulation in the margine mollusc Aplysia. Physiological concentrations of dopamine (diluted in seawater) were perfused through the gill during siphon stimulation series. The amplitude of the reflex was potentiated by dopamine and habituation of the reflex was prevented. This occurred with no change in the activity evoked in central motor neurons. These results lead us to conclude that the dopaminergic motor neuron L9 is modulating habituation in the periphery and that the central nervous system facilitatory control of the peripheral nervous system may act via a dopaminergic pathway.


2018 ◽  
Vol 20 (4) ◽  
pp. 439-441
Author(s):  
Jairo Silva Dos Ângelos ◽  
Guilherme Brasileiro De Aguiar ◽  
Edgar Manuel Garcete Fariña ◽  
Maud Parise ◽  
Alexandre Martins Cunha ◽  
...  

Sarcoidosis is a disease of unknown etiology presenting as noncaseating granulomatous infiltration in tissues. Central Nervous system is rarely affected , usually as dissemination from other organs, mainly lungs. We report on a case in which the patient had only central nervous system involvement, presenting with a mass in the right gasserian ganglion and another lesion in right parietal cortex, observed in magnetic resonance. Diagnosis was given by histopathological study of the lesion and the patient improved with corticosteroid treatment.


1983 ◽  
Vol 61 (7) ◽  
pp. 743-748 ◽  
Author(s):  
Ken Lukowiak ◽  
Lee Freedman

In Aplysia, the central nervous system and peripheral nervous system interact and form an integrated system that mediates adaptive gill withdrawal reflex behaviours evoked by tactile stimulation of the siphon. The central nervous system (CNS) exerts suppressive and facilitatory control over the peripheral nervous system (PNS) in the mediation of these behaviours. We found that the CNS's suppressive control over the PNS was increased significantly in animals engaged in sexual activity as either a male or female. In control animals, the evoked gill withdrawal reflex met a minimal response amplitide criterion, while in sexually active animals the reflex did not meet this criterion. At the neuronal level, the increased CNS suppressive control was manifested as a decrease in excitatory input to the central gill motor neurons.


1985 ◽  
Vol 100 (1) ◽  
pp. 74-85 ◽  
Author(s):  
S C Papasozomenos ◽  
L I Binder ◽  
P K Bender ◽  
M R Payne

We have examined the distribution of microtubule-associated protein 2 (MAP2) in the lumbar segment of spinal cord, ventral and dorsal roots, and dorsal root ganglia of control and beta,beta'-iminodipropionitrile-treated rats. The peroxidase-antiperoxidase technique was used for light and electron microscopic immunohistochemical studies with two monoclonal antibodies directed against different epitopes of Chinese hamster brain MAP2, designated AP9 and AP13. MAP2 immunoreactivity was present in axons of spinal motor neurons, but was not detected in axons of white matter tracts of spinal cord and in the majority of axons of the dorsal root. A gradient of staining intensity among dendrites, cell bodies, and axons of spinal motor neurons was present, with dendrites staining most intensely and axons the least. While dendrites and cell bodies of all neurons in the spinal cord were intensely positive, neurons of the dorsal root ganglia were variably stained. The axons of labeled dorsal root ganglion cells were intensely labeled up to their bifurcation; beyond this point, while only occasional central processes in dorsal roots were weakly stained, the majority of peripheral processes in spinal nerves were positive. beta,beta'-Iminodipropionitrile produced segregation of microtubules and membranous organelles from neurofilaments in the peripheral nervous system portion and accumulation of neurofilaments in the central nervous system portion of spinal motor axons. While both anti-MAP2 hybridoma antibodies co-localized with microtubules in the central nervous system portion, only one co-localized with microtubules in the peripheral nervous system portion of spinal motor axons, while the other antibody co-localized with neurofilaments and did not stain the central region of the axon which contained microtubules. These findings suggest that (a) MAP2 is present in axons of spinal motor neurons, albeit in a lower concentration or in a different form than is present in dendrites, and (b) the MAP2 in axons interacts with both microtubules and neurofilaments.


2008 ◽  
Vol 21 (6) ◽  
pp. 810-816 ◽  
Author(s):  
F.A. Al Hajri ◽  
A.T. Muqim ◽  
T.J.E. Muttikkal

Sarcoidosis is a chronic multi-system granulomatous disorder of unknown etiology. Central nervous system involvement is relatively uncommon in sarcoidosis. Clinical manifestations and radiological appearances of neurosarcoidosis vary widely depending on the site and activity of the lesions. In most cases, the imaging appearance is nonspecific. We report a very rare case of extensive neurosarcoidosis with progressively enlarging cystic lesions in the right temporal lobe.


Author(s):  
S.S. Spicer ◽  
B.A. Schulte

Generation of monoclonal antibodies (MAbs) against tissue antigens has yielded several (VC1.1, HNK- 1, L2, 4F4 and anti-leu 7) which recognize the unique sugar epitope, glucuronyl 3-sulfate (Glc A3- SO4). In the central nervous system, these MAbs have demonstrated Glc A3-SO4 at the surface of neurons in the cerebral cortex, the cerebellum, the retina and other widespread regions of the brain.Here we describe the distribution of Glc A3-SO4 in the peripheral nervous system as determined by immunostaining with a MAb (VC 1.1) developed against antigen in the cat visual cortex. Outside the central nervous system, immunoreactivity was observed only in peripheral terminals of selected sensory nerves conducting transduction signals for touch, hearing, balance and taste. On the glassy membrane of the sinus hair in murine nasal skin, just deep to the ringwurt, VC 1.1 delineated an intensely stained, plaque-like area (Fig. 1). This previously unrecognized structure of the nasal vibrissae presumably serves as a tactile end organ and to our knowledge is not demonstrable by means other than its selective immunopositivity with VC1.1 and its appearance as a densely fibrillar area in H&E stained sections.


Author(s):  
Konstantin Gulyabin

Mills' syndrome is a rare neurological disorder. Its nosological nature is currently not completely determined. Nevertheless, Mills' syndrome is considered to be a rare variant of the degenerative pathology of the central nervous system – a variant of focal cortical atrophy. The true prevalence of this pathology is unknown, since this condition is more often of a syndrome type, observed in the clinical picture of a number of neurological diseases (primary lateral sclerosis, frontotemporal dementia, etc.) and is less common in isolated form.


Sign in / Sign up

Export Citation Format

Share Document