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*

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*

Postganglionic cholinergic dysautonomia is a rare disease characterized by impaired secretion of tears and saliva, generalized absence of sweating, lack of cardiac response to carotid massage and atony of the gastrointestinal tract and urinary bladder. Autonomic function tests show the preservation of sympathetic adrenergic functions in contrast to the generalized involvement of postganglionic parasympathetic and sympathetic cholinergic nerves. Recently, we experienced a patient with gut dysmotility in selective postganglionic cholinergic dysautonomia. She is now being treated with bethanechol and milk of magnesia and has experienced minimal symptom improvement.
Bethanechol ; Gastrointestinal Tract ; Humans ; Magnesium Oxide ; Massage ; Milk ; Primary Dysautonomias* ; Rare Diseases ; Saliva ; Sweat ; Sweating ; Tears ; Urinary Bladder

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

BACKGROUND AND PURPOSE: Excessive daytime sleepiness and sudden sleep attacks are the main features of narcolepsy, but rapid-eye-movement sleep behavior disorder (RBD), hyposmia, and depression can also occur. The latter symptoms are nonmotor features in idiopathic Parkinson's disease (IPD). In the present study, IPD-proven diagnostic tools were tested to determine whether they are also applicable in the assessment of narcolepsy. METHODS: This was a case-control study comparing 15 patients with narcolepsy (PN) and 15 control subjects (CS) using the Scales for Outcomes in Parkinson's Autonomic Test (SCOPA-AUT), Parkinson's Disease Nonmotor Symptoms (PDNMS), University of Pennsylvania Smell Test, Farnsworth-Munsell 100 Hue test, Beck Depression Inventory, and the RBD screening questionnaire. RESULTS: Both the PN and CS exhibited mild hyposmia and no deficits in visual tests. Frequent dysautonomia in all domains except sexuality was found for the PN. The total SCOPA-AUT score was higher for the PN (18.47+/-10.08, mean+/-SD) than for the CS (4.40+/-3.09), as was the PDNMS score (10.53+/-4.78 and 1.80+/-2.31, respectively). RBD was present in 87% of the PN and 0% of the CS. The PN were more depressed than the CS. The differences between the PN and CS for all of these variables were statistically significant (all p<0.05). CONCLUSIONS: The results of this study provide evidence for the presence of dysautonomia and confirm the comorbidities of depression and RBD in narcolepsy patients. The spectrum, which is comparable to the nonmotor complex in IPD, suggests wide-ranging, clinically detectable dysfunction beyond the narcoleptic core syndrome.
Case-Control Studies ; Comorbidity ; Depression ; Humans ; Mass Screening ; Narcolepsy* ; Parkinson Disease ; Pennsylvania ; Primary Dysautonomias* ; Sexuality ; Smell ; Weights and Measures ; Surveys and Questionnaires

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (¹⁸F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in ¹⁸F-FDG PET.
Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; Diagnosis ; Gyrus Cinguli ; Humans ; Neuroimaging ; Positron-Emission Tomography ; Primary Dysautonomias ; Thalamus ; Vestibular Neuronitis

The causes of sudden death after medullary infarction involve arrhythmia, central respiratory failure, and dysautonomia. Sudden cardiac arrest in a medullary infarction is uncommon. Most of these cases experienced sudden cardiopulmonary arrest within 2 weeks from stroke onset as the extent of lesion increased. Here, we report two cases of medullary infarction presenting as sudden cardiac arrest. These cases indicate that medullary infarction could be one of the causes of sudden cardiac arrest.
Arrhythmias, Cardiac ; Death, Sudden ; Death, Sudden, Cardiac ; Heart Arrest ; Infarction ; Medulla Oblongata ; Primary Dysautonomias ; Respiratory Insufficiency ; Stroke