Multiple system atrophy (MSA) is an idiopathic neurodegenerative disorder involving many neuronal structures. It is a heterogeneous system disorder affecting extrapyramidal, cerebellar and autonomic nervous system. Only a minority of MSA patients are diagnosed before reaching the full blown stage. Its autonomic features like orthostatic hypotension, vocal cord paralysis are directly related to mortality. Up to now, rehabilitation of MSA patients had been rarely reported. Early, proper diagnosis and comprehensive rehabilitation for their heterogeneous clinical features are important. We experienced 54 year old hemiplegic paient who overlaps MSA. He showed some improvement in ADL and cerebellar symptoms after comprehensive rehabilitation programs.
Activities of Daily Living ; Autonomic Nervous System ; Diagnosis ; Humans ; Hypotension, Orthostatic ; Middle Aged ; Mortality ; Multiple System Atrophy* ; Neurodegenerative Diseases ; Neurons ; Rehabilitation ; Stroke* ; Vocal Cord Paralysis

Dysfunction of the autonomic nervous system is reported to occur at an incidence of 20% to 40% in diabetes. The clinical symptoms include orthostatic hypotension, vomiting, diarrhea, bladder dysfunction, male impotence, sweating, etc. Two simple noninvasive tests, sympathetic skin response (SSR) and R-R interval variation (RRIV), were used to assess autonomic functions. We performed SSR and RRIV on the diabetic patients and controls. The patients were classified into 4 groups (group I: without peripheral neuropathy or dysautonomia, group II: with dysautonomia only, group III: with peripheral neuropathy only, group IV: with both peripheral neuropathy and dysautonomia). We also tried to correlate their clinical dysautonomic symptoms and the results of nerve conduction studies (NCS) and of SSR and RRIV. The subjects of this study were 82 diabetic patients, 20 to 73 years old with the mean age of 53, and 12 controls. Latency, amplitude, and loss of SSR all showed a significant difference in relation to the dysautonomic symptoms. The loss of SSR in the foot showed a remarkable difference in group I. In groups III and IV, three RRIVs (Valsalva ratio, E:I ratio, 30 : 15 ratio) showed a significant decrease compared with the control group, and in group II, only the 30:15 ratio showed a statistically significant decrease. In conclusion, the changes in SSR and RRIV were significantly associated with the dysautonomia. Among these, loss of SSR in the foot and decrease in the 30 : 15 ratio were useful parameters for early detection of diabetic autonomic neuropathy without peripheral neuropathy.
Aged ; Autonomic Nervous System ; Diabetic Neuropathies ; Diarrhea ; Erectile Dysfunction ; Foot ; Humans ; Hypotension, Orthostatic ; Incidence ; Male ; Neural Conduction ; Peripheral Nervous System Diseases ; Primary Dysautonomias ; Skin ; Sweat ; Sweating ; Urinary Bladder ; Vomiting

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.
Aneurysm ; Anisocoria ; Autonomic Nervous System ; Constriction ; Fistula ; Horner Syndrome ; Iris ; Nervous System Diseases ; Pupil ; Pupil Disorders ; Tonic Pupil

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.
Aneurysm ; Anisocoria ; Autonomic Nervous System ; Constriction ; Fistula ; Horner Syndrome ; Iris ; Nervous System Diseases ; Pupil ; Pupil Disorders ; Tonic Pupil

BACKGROUND & OBJECT10NS: Multiple system atrophy(MSA) is a heterogenous system disorder affecting extrapyramidal, cerebellar and autonomic nervous system. Clinical spectrum is broad, and depending on the system affected, patients are classified into striato-nigral degeneration (SND), olivo-ponto-cerebellar atrophy (OPCA) and Shy-Draper syndrome (SDS). However, evolution of symptoms during follow-up usually occurs, stirring up a debate between "lumpers" and "splitters". Recent pathological documentation of intracytoplasmic inclusions support "lumpers" that MSA is a specific disease entity with specific pathology. The study was done to analyze the natural course of MSA, and examine whether they are separate or part of the same disease. METHOD: We obtained the clinical data of patients with clinically probable MSA by the criteria of Quinn (1994). In addition to review of medical records, all patients were phone-interviewed or examined personally. RESULTS: Forty four patients were included in the study (male 23, female 21). Mean onset age 52.9 years, and mean follow-up period 19.7 months. Nine patients died during follow-up (mean disease duration 5.2 years). The initial predominant features were parkinsonism in 40% (14/35), cerebellar dysfunction in 25.7% (9/35), autonomic dysfunction in 17.1% (6/35) and others in 17.1%. At the latest follow-up, parkinsonism were noted in 77.1%, cerebellar dysfunction in 88.6% and autonomic dysfunction in 80%. With progression, all the patients showed mixed clinical manifestations, the most common being combination of all 3(60%). CONCLUS10N: The data supports that SND, OPCA and SDS are part of the same disease process.
Age of Onset ; Autonomic Nervous System ; Cerebellar Diseases ; Female ; Follow-Up Studies ; Humans ; Medical Records ; Natural History* ; Olivopontocerebellar Atrophies ; Parkinsonian Disorders ; Pathology

Multiple system atrophy (MSA) is a rare, yet fatal neurodegenerative disease that presents clinically with autonomic failure in combination with parkinsonism or cerebellar ataxia. MSA impacts on the autonomic nervous system affecting blood pressure, heart rate and bladder function, and the motor system affecting balance and muscle movement. The cause of MSA is unknown, no definitive risk factors have been identified, and there is no cure or effective treatment. The definitive pathology of MSA is the presence of alpha-synuclein aggregates in the brain and therefore MSA is classified as an alpha-synucleinopathy, together with Parkinson's disease and dementia with Lewy bodies. Although the molecular mechanisms of misfolding, fibrillation and aggregation of alpha-synuclein partly overlap with other alpha-synucleinopathies, the pathological pathway of MSA is unique in that the principal site for alpha-synuclein deposition is in the oligodendrocytes rather than the neurons. The sequence of pathological events of MSA is now recognized as abnormal protein redistributions in oligodendrocytes first, followed by myelin dysfunction and then neurodegeneration. Oligodendrocytes are responsible for the production and maintenance of myelin, the specialized lipid membrane that encases the axons of all neurons in the brain. Myelin is composed of lipids and two prominent proteins, myelin basic protein and proteolipid protein. In vitro studies suggest that aberration in protein distribution and lipid transport may lead to myelin dysfunction in MSA. The purpose of this perspective is to bring together available evidence to explore the potential role of alpha-synuclein, myelin protein dysfunction, lipid dyshomeostasis and ABCA8 in MSA pathogenesis.
alpha-Synuclein ; Autonomic Nervous System ; Axons ; Blood Pressure ; Brain ; Cerebellar Ataxia ; Dementia ; Heart Rate ; Lewy Bodies ; Membranes ; Multiple System Atrophy* ; Myelin Proteins ; Myelin Sheath* ; Neurodegenerative Diseases ; Neurons ; Oligodendroglia ; Parkinson Disease ; Parkinsonian Disorders ; Pathology ; Risk Factors ; Urinary Bladder

Acute Disease ; Autonomic Nervous System Diseases ; Child ; Humans ; Male

Autonomic Nervous System Diseases ; physiopathology ; Humans ; Liver Cirrhosis ; physiopathology

Near-infrared spectroscopy (NIRS), a noninvasive optical method, utilizes the characteristic absorption spectra of hemoglobin in the near-infrared range to provide information on cerebral hemodynamic changes in various clinical situations. NIRS monitoring have been used mainly to detect reduced perfusion of the brain during orthostatic stress for three common forms of orthostatic intolerance (OI); orthostatic hypotension, neurally mediated syncope, and postural orthostatic tachycardia syndrome. Autonomic function testing is an important diagnostic test to assess their autonomic nervous systems for patients with symptom of OI. However, these techniques cannot measure dynamic changes in cerebral blood flow. There are many experimentations about study of NIRS to reveal the pathophysiology of patients with OI. Research using NIRS in other neurologic diseases (stroke, epilepsy and migraine) are ongoing. NIRS have been experimentally used in all stages of stroke and may complement the established diagnostic and monitoring tools. NIRS also provide pathophysiological approach during rehabilitation and secondary prevention of stroke. The hemodynamic response to seizure has long been a topic for discussion in association with the neuronal damage resulting from convulsion. One critical issue when unpredictable events are to be detected is how continuous NIRS data are analyzed. Besides, NIRS studies targeting pathophysiological aspects of migraine may contribute to a deeper understanding of mechanisms relating to aura of migraine. NIRS monitoring may play an important role to trend regional hemodynamic distribution of flow in real time and also highlights the pathophysiology and management of not only patients with OI symptoms but also those with various neurologic diseases.
Absorption ; Autonomic Nervous System ; Brain ; Cerebrovascular Circulation ; Complement System Proteins ; Diagnostic Tests, Routine ; Epilepsy ; Hemodynamics ; Humans ; Hypotension, Orthostatic ; Methods ; Migraine Disorders ; Nervous System Diseases* ; Neurons ; Orthostatic Intolerance* ; Perfusion ; Postural Orthostatic Tachycardia Syndrome ; Rehabilitation ; Secondary Prevention ; Seizures ; Spectroscopy, Near-Infrared* ; Spectrum Analysis ; Stroke ; Syncope

Heart rate variability is significantly associated with cardiovascular complications in various neurological disorders with cardiac impairment. Measures of spontaneous heart rate variability might be different from provocating tests of heart rate variability such as deep breathing and Valsava maneuver. Methods for analysis are divided into time domain methods and frequency domain methods. There are standard deviation of NN interval, standard deviation of average NN interval, root mean square of the successive differences, NN50, and pNN50 in time domain methods. Frequency domain bands can be divided into very low, low, and high frequency. Each variables are influenced by sympathetic and/or parasympathetic activity.
Autonomic Nervous System ; Cardiovascular Abnormalities ; Heart Rate* ; Nervous System Diseases ; Respiration