BACKGROUND AND PURPOSE: Parkinson's disease (PD) and multiple-system atrophy of the parkinsonian type (MSA-P) are progressive neurodegenerative disorders that in addition to dysfunction of the motor system also present with features of dysautonomia, frequently manifesting as orthostatic hypotension (OH). The pathophysiology of OH has been proposed to differ between these two disorders. This study investigated the spontaneous and cardiovagal baroreflex sensitivity (BRS) in Parkinson's disease patients with orthostatic hypotension (PD(OH)) and multiple system atrophy of Parkinsonian type with orthostatic hypotension in an attempt to differentiate the two disorders. METHODS: Two methods were used for determining the BRS: a spontaneous method (spontaneous BRS) and the reflexive baroreflex gain (cardiovagal BRS) from phases II and IV of the Valsalva maneuver (VM) in PD(OH) and MSA-P(OH). RESULTS: The spontaneous BRS (5.04±0.66 ms/mm Hg vs. 4.78±0.64 ms/mm Hg, p=0.54) and the cardiovagal BRS from phase II of the VM (0.96±0.75 ms/mm Hg vs. 1.34±1.51 ms/mm Hg, p=0.76) did not differ between PD(OH) and MSA-P(OH), but the cardiovagal BRS from phase IV of the VM (0.03±0.07 ms/mm Hg vs. 2.86±2.39 ms/mm Hg, p=0.004) was significantly lower in PD(OH). CONCLUSIONS: The cardiovagal BRS from phase IV of the VM has potential for differentiating PD(OH) and MSA-P(OH), indicating a difference in the pathophysiological mechanisms underlying the autonomic dysfunction in the two disorders.
Atrophy* ; Baroreflex* ; Humans ; Hypotension, Orthostatic ; Multiple System Atrophy ; Neurodegenerative Diseases ; Parkinson Disease* ; Primary Dysautonomias ; Reflex ; Valsalva Maneuver

Familial dysautonomia, first described by Riley and co-workers in 1949, is a congenital, heritable syndrome. It is transmitted by a recessive autosomal gene which is generally limited to persons of Jewish extraction; however, rare occurrences among non-Jewish Caucasians and in a black girl have been reported. An eight-year old Korean boy was admitted to Severance Hospital with bulbar conjunctival injection OU for 1 month and visual disturbance OS for 10 days. Examination revealed exfoliated epithelium in an area of about 3mm in diameter in the center of the left cornea, multiple punctate erosions and edema of the corneal epithelium. Yet, he experienced no discomfort, blepharospasm, nor photophobia. He had decreased corneal sensation, decreased lacrimation by Schirmer test and miosis was induced biJaterally by 0.25% pilocarpine (equivalent to 2.5% methacholine). The patient had decreased deep tendon reflexes and postural hypotension, and showed emotional lability, indifference to pain, and abnormal temperature control. He also had marked anorexia, swallowing difficulty, cyclic vomiting, abdominal pain, headache, intermittent vascular hypertension and one episode of convulsive seizure. He was positive to histamine intradermal injection test and had abnormal EEG. EMG was suggestive of some form of neuropathy. He was treated with toplca 0.5% chloramphenicol solution and 10% dextran solution alternatively q.2 hrs., terramycin ophthalmic ointment q.h.s. and bilateral patching; he also received 50,000 units of vitamin A for 10 days. He showed marked improvement of his ocular symptoms in 6 days. The above patient shows many of the essential features of the familial dysautonomia syndrome as outlined by Riley; however, he also lacks some of the most important features. Therefore, we feel that the patient has a case of some other type of autonomic dysfunction simulating Riley-Day svndrome.
Abdominal Pain ; Anorexia ; Blepharospasm ; Chloramphenicol ; Cornea ; Deglutition ; Dextrans ; Dysautonomia, Familial* ; Edema ; Electroencephalography ; Epithelium ; Epithelium, Corneal ; Female ; Headache ; Histamine ; Humans ; Hypertension ; Hypotension, Orthostatic ; Injections ; Injections, Intradermal ; Male ; Miosis ; Oxytetracycline ; Photophobia ; Pilocarpine ; Primary Dysautonomias* ; Reflex, Stretch ; Seizures ; Sensation ; Vitamin A ; Vomiting

Modern autonomic function tests can non-invasively evaluate the severity and distribution of autonomic failure. They have sufficient sensitivity to detect even subclinical dysautonomia. Standard laboratory testing evaluates cardiovagal, sudomotor and adrenergic autonomic functions. Cardiovagal function is typically evaluated by testing heart rate response to deep breathing at a defined rate and to the Valsalva maneuver. Sudomotor function can be evaluated with the quantitative sudomotor axon reflex test and the thermoregulatory sweat test. Adrenergic function is evaluated by the blood pressure and heart rate responses to the Valsalva maneuver and to head-up tilt. Tests are useful in defining the presence of autonomic failure, their natural history, and response to treatment. They can also define patterns of dysautonomia that are useful in helping the clinician diagnose certain autonomic conditions. For example, the tests are useful in the diagnosis of the autonomic neuropathies and distal small fiber neuropathy. The autonomic neuropathies (such as those due to diabetes or amyloidosis) are characterized by severe generalized autonomic failure. Distal small fiber neuropathy is characterized by an absence of autonomic failure except for distal sudomotor failure. Selective autonomic failure (which only one system is affected) can be diagnosed by autonomic testing. An example is chronic idiopathic anhidrosis, where only sudomotor function is affected. Among the synucleinopathies, autonomic function tests can distinguish Parkinson's disease (PD) from multiple system atrophy (MSA). There is a gradation of autonomic failure. PD is characterized by mild autonomic failure and a length-dependent pattern of sudomotor involvement. MSA and pure autonomic failure have severe generalized autonomic failure while DLB is intermediate.
Axons ; Blood Pressure ; Heart Rate ; Hypohidrosis ; Multiple System Atrophy ; Natural History ; Parkinson Disease ; Primary Dysautonomias ; Pure Autonomic Failure ; Reflex ; Respiration ; Sweat ; Valsalva Maneuver

The clinical and urodynarnic studies for urinary dysfunction were performed in 35 patients with idiopathic parkinson's disease who admitted at Hanyang University Hospital from January, 1988 to June, 1990. The results are as follows; 1. Urinary dysfunction was noted in 9 patients (25.7%) with idiopathic parkinson's disease Their cysto / uroflowmetry showed detrusor hyperreflexia in 6 patients, and detrusor hyporeflexia in 3 patients. The common urinary symptoms were frequency, incontinence, hesitency, decreased urinary force, and retention in that order. 2. The mean time-interval from the onset of initial parkinsonian symptoms to the development of urinary symptoms was 22.6 months (ranged from 3 to 48 months). Urinary dysfuncion showed no correlation with clinical severity of parkinson's disease. 3. The frequency of urinary dysfunction was greater in rigidity-predominant parkinsonian group than patients with tremor. Cysto / uroflowmetry in the former showed detrusor hyperreflexia more frequently than detrusor hyporeflexia. 4. It was suggested that urinary dysfunction in idiopathic parkinson's disease probably result from multifactorial causes such as the change of neuronal effect, dysautonomia, and skeletal rigidity.
Humans ; Neurons ; Parkinson Disease* ; Primary Dysautonomias ; Reflex, Abnormal ; Tremor

Humans ; Mutation ; Primary Dysautonomias/etiology* ; Sleep Initiation and Maintenance Disorders/etiology*

Dysfunction of autonomic nervous system had been reported in a number of neurologic and medical diseases, but acquired primary dysautonomia is uncommon and usually tends to show a chronic progressive pattern. Acute panautonomic neuropathy characterized by selective involvement of sympathetic and parasympathetic nerves with complete or relative sparing of somatic motor and sensory functions was first described by Young et. Al, in 1969 and since then, about 26 additional cases had been reported in the literatures, but there have been no such reports in Korea. The present report describes a 22 year-old female patient who had been admitted to Severance hospital because of abruptly developed fever, generalized myalgia, hyperesthesia and difficulties in swallowing and voiding. On admission, physical and neurological examinations revealed orthostatic hypotension, unreactive pupils to light and accomodation, dereased gag reflex, and decreased sensation of pain and temperature on the face and neck. A few days following admission, progressive loss of deep tendon reflew was noticed, followed by distal muscle weakness and atrophy. The serial electrophysiologic studies revealed progressive peripheral axonal polyneuropathy. Autonomic function tests suggested pandysautonomia of postganglionic lesion. Spinal fluid protein was 16mg/dl on admission, but was increased up to 80mg/dl without pleocytosis on the twenty first hospital day. Recovery was poor over a four -month period and she died of disseminated pneumonia.
Atrophy ; Autonomic Nervous System ; Axons ; Deglutition ; Female ; Fever ; Humans ; Hyperesthesia ; Hypotension, Orthostatic ; Korea ; Leukocytosis ; Muscle Weakness ; Myalgia ; Neck ; Neurologic Examination ; Pneumonia ; Polyneuropathies ; Primary Dysautonomias ; Pupil ; Reflex ; Sensation ; Tendons ; Young Adult

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

Acute panautonomic neuropathy characterized by selective involvement of sympathetic and parasympathetic nerves with complete or relative sparing of somatic motor and sensory functions was first described by Young et al in 1969. We experienced acute pandysautonomia with peripheral neuropathy. The serial electrophysiologic studies revealed progressive periphear axonal polyneuropathy. Autonomic function test suggested dysautonomia of selective postganglionic lesion in 1 case and pandysautonomia in 3 cases. Our four cases wree Acute panautonomic neuropathies which simultaneously developed sensorimotor polyneuropathy. All Laps were normal except elevated CSF protein without pleocytosis. Recovery was poor in general, but 34-years-old female patient was markedly improved after plasmapheresis. Therefore we recommend immunologic treatment such as plasmapheresis or pulse therapy in early stage of progressive pandysautonomia.
Axons ; Female ; Humans ; Leukocytosis ; Peripheral Nervous System Diseases ; Plasmapheresis ; Polyneuropathies ; Primary Dysautonomias ; Sensation

Myokymia is one of involuntary movement, which is characterized by undulatory muscle spasm, similar to the worm's crawl. Sometimes muscle pain, itchy sensation, dysautonomia and other symptoms are associated with it. Derangement of the peripheral or central nervous system after nerve or tissue damage is suspected as the source of impulse generators causing this symptom. We encountered a patient with neuropathic pain and myokymia after thoracotomy. Although several medications and nerve blocks have been applied, all have failed to provide symptom relief. We experienced improvement of the pain and involuntary movement with a thoracic sympathetic ganglion block and gabapentin.
Central Nervous System ; Dyskinesias ; Ganglia, Sympathetic* ; Humans ; Myalgia ; Myokymia* ; Nerve Block ; Neuralgia* ; Primary Dysautonomias ; Sensation ; Spasm ; Thoracotomy*

We present a case of an 8-year-old boy with visceral autonomic neuropathy complicated by Guillain-Barre syndrome. In this pediatric patient, gastroparesis was the major symptom among the autonomic symptoms. Due to the gastroparesis, there was no progress with the oral diet, and nutrition was therefore supplied through a nasojejunal tube and gastrojejunal tube via Percutaneous endoscopic gastrostomy (PEG). After tube feeding for 9 months, the patient's gastrointestinal symptoms improved and his oral ingestion increased. The pediatric patient was maintained well without gastrointestinal symptoms for 3 months after removal of the PEG, had repeated vomiting episodes which lead to the suspicion of cyclic vomiting syndrome. Then he started treatment with low-dose amitriptyline, which resulted in improvement. Currently, the patient has been maintained well for 6 months without recurrence, and his present growth status is normal.
Amitriptyline ; Child* ; Diet ; Eating ; Enteral Nutrition ; Gastroparesis ; Gastrostomy ; Guillain-Barre Syndrome* ; Humans ; Male ; Primary Dysautonomias ; Recurrence ; Vomiting*