Consensus paper: management of degenerative cerebellar disorders.

Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult. Yet there are recent developments that are likely to lead to significant improvements in the future. Most desirable would be a causative treatment of the underlying cerebellar disease. This is currently available only for a very small subset of cerebellar ataxias with known metabolic dysfunction. However, increasing knowledge of the pathophysiology of hereditary ataxia should lead to an increasing number of medically sensible drug trials. In this paper, data from recent drug trials in patients with recessive and dominant cerebellar ataxias will be summarized. There is consensus that up to date, no medication has been proven effective. Aminopyridines and acetazolamide are the only exception, which are beneficial in patients with episodic ataxia type 2. Aminopyridines are also effective in a subset of patients presenting with downbeat nystagmus. As such, all authors agreed that the mainstays of treatment of degenerative cerebellar ataxia are currently physiotherapy, occupational therapy, and speech therapy. For many years, well-controlled rehabilitation studies in patients with cerebellar ataxia were lacking. Data of recently published studies show that coordinative training improves motor function in both adult and juvenile patients with cerebellar degeneration. Given the well-known contribution of the cerebellum to motor learning, possible mechanisms underlying improvement will be outlined. There is consensus that evidence-based guidelines for the physiotherapy of degenerative cerebellar ataxia need to be developed. Future developments in physiotherapeutical interventions will be discussed including application of non-invasive brain stimulation.

Clarification of muscle synergy structure during standing-up motion of healthy young, elderly and post-stroke patients.

Standing-up motion is an important daily activity. It has been known that elderly and post-stroke patients have difficulty in performing standing-up motion. The standing-up motion is retrained by therapists to maximize independence of the elderly and post-stroke patients, but it is not clear how the elderly and post-stroke patients control their redundant muscles to achieve standing-up motion. This study employed the concept of muscle synergy to analyze how healthy young adults, healthy elderly people and post-stroke patients control their muscles. Experimental result verified that four muscle synergies can represent human standing-up motion. In addition, it indicated that the post-stroke patients shift the weights of muscle synergies to finish standing-up motion comparing to healthy subjects. Moreover, different muscle synergy structures were associated with the CoM and joint kinematics.

Patients with stroke confined to basal ganglia have diminished response to rehabilitation efforts.

Prediction of the functional outcome for patients with stroke has depended on the severity of impairment, location of brain injury, age, and general medical condition. This study compared admission and discharge functional outcome (Functional Independence Measure, FIM) and deficit severity (Fugl-Meyer, F-M) scores in a retrospective study of patients with similar neurologic impairments: homonymous hemianopia, hemisensory loss, and hemiparesis. CT-verified stroke location was the independent variable: cortical (n = 11), basal ganglia and internal capsule (normal cortex and thalamus, n = 13), or combined (cortical, basal ganglia, and internal capsule, n = 22). By 3 months on average after stroke, all groups demonstrated significantly improved motor function as measured by F-M scores. Patients with cortical lesions had the least CT-imaged damage and the best outcome. Patients with combined lesions and more extensive brain injury had significantly higher FIM scores (P < 0.05) than patients with injury restricted to the basal ganglia/ internal capsule. Patients with basal ganglia/internal capsule injury were more likely to have hypotonia, flaccid paralysis, and persistently impaired balance and ambulation performance. While all patients had a comparable rehabilitation experience, these results suggest that patients with stroke confined to the basal ganglia and internal capsule benefited less from therapy. Isolated basal ganglia stroke may cause persistent corticothalamic-basal ganglia interactions that are dysfunctional and impede recovery.

Wallerian degeneration of the pyramidal tract does not affect stroke rehabilitation outcome.

OBJECTIVE: To test whether Wallerian degeneration (WD) of the pyramidal tract as signaled by MRI affects rehabilitation outcome in patients with subcortical infarction (internal capsule or corona radiata). BACKGROUND: Recent radiologic evidence suggests that WD occurs no earlier than 3 months after a subcortical infarction. METHODS: A total of 77 consecutive patients with pure motor hemiparesis due to an initial subcortical infarction were assessed on admission and discharge with the Functional Independence Measure (FIM) for disability and Stroke Impairment Assessment Set (SIAS, full = 25) for impairment. WD was defined by a high-intensity area detected along the pyramidal tract below the level of lesion on T2-weighted MR image (WD+). RESULTS: Age, sex, side of stroke, Mini-Mental State Examination score, and volume of lesion were comparable for each group. Length of stay (LOS) was significantly longer (p < 0.05) in WD+ (130 days) than in WD- (105 days). There was no difference in the change of FIM (WD+, 99 to 111; WD-, 95 to 107) or SIAS measures (WD+, 12 to 16; WD-, 13 to 16) made on admission and discharge, nor was there any effect of the timing of the rehabilitation experience (< or = 90 days or >90 days after stroke). CONCLUSIONS: After stroke, apparent WD of the pyramidal tract may slow functional recovery but does not limit final rehabilitation outcome of pure motor hemiparesis. Study of the mechanisms of compensation for this delayed pyramidal tract degeneration will enhance the scientific basis for rehabilitation.

A pilot study of the effect of L-threodops on rehabilitation outcome of stroke patients.

We investigated whether L-threodops (L-DOPS), a norepinephrine precursor, improves rehabilitation outcome in patients with initial hemiparetic supratentorial ischemic stroke (2 months post stroke). Five patients who agreed to be treated with L-DOPS received 45-minute physical therapy (PT) and occupational therapy (OT) for 2 months, 3 days a week, with an oral dose of 200 mg L-DOPS 2 hours before each session, followed by PT and OT without L-DOPS for 2 months (DOPS group). Eight patients who disagreed received PT and OT for 4 months (control). Each group demonstrated comparable age, sex, complications, Mini-Mental State Examination, and the baseline Functional Independence Measure (FIM, DOPS/control = 36/42), Fugl-Meyer (F-M) motor scale (30/27), and ambulation endurance (10/9 meters). DOPS group had significantly greater gain than control (p < 0.05, Mann-Whitney U test) in FIM score at 4 (51/45) and 6 months (57/49), ambulation at 4 (66/16) and 6 months (82/24), and F-M score at 4 months (40/29). There were no side effects that required discontinuation of the drug. These results suggest that L-DOPS treatment paired with PT and OT may be effective in improving functional outcome in stroke.

Patients with capsular infarct and Wallerian degeneration show persistent regional premotor cortex activation on functional magnetic resonance imaging.

BACKGROUND AND PURPOSE: By using neurorehabilitation outcome measures and functional magnetic resonance imaging (fMRI), we attempted to elucidate the effect of Wallerian degeneration (WD) in the pyramidal tract distal to a posterior capsular stroke on functional recovery. METHODS: In 18 patients with pure motor hemiparesis caused by capsular infarct, we identified the presence of WD and then tested whether it affected the rate of motor improvement and the final motor outcome. The discharge T2-weighted MRI (139 +/- 5 days on average after stroke) showed WD in 10 of 18 patients (WD-positive, n = 10; WD-negative, n = 8). All patients performed mass grasping of paretic fingers before and after inpatient neurorehabilitation for the fMRI. RESULTS: Demographic characteristics, rate of disability change, final motor status, and volume of lesion were comparable between the groups. On the first fMRI, patterns of fMRI activation in the sensorimotor cortex, premotor cortex (PMC), and supplementary motor area were comparable. However, on the second fMRI, considerably more patients in the WD-positive group (8 out of 10) exhibited persistent contralateral activation in PMC than in the WD-negative group (1 out of 8; P = .0044, chi-square test). Ipsilateral PMC was also more frequently activated (P = .04) in WD-positive patients than in WD-negative patients. CONCLUSIONS: Persistent WD had no effect on the impairment or disability outcome; however, it was associated with novel regional activation on repeat fMRI after recovery. To determine whether persistent PMC activation resulted from effort or represents a general effect of WD on motor recovery will require a longer follow-up time and more precise control of functional measurement during imaging.

[Intra-abdominal visceral fat in myotonic dystrophy].

We first clarified the relationship between abdominal visceral fat accumulation and impaired glucose and lipid metabolism in patients with myotonic dystrophy. Nineteen patients aged 44.2 +/- 11.4 years with a body mass index (BMI) of 20.5 +/- 2.8 kg/m2, and 18 controls aged 45.2 +/- 10.3 years, with a BMI 20.0 +/- 2.8 kg/m2 were examined. The distribution of abdominal visceral and subcutaneous fat was examined by CT scanning at the umbilical level. Visceral fat area was significantly greater in myotonic dystrophy than that in control (p < 0.05), and positively correlated with the levels of fasting plasma glucose (r = 0.65 p < 0.01), plasma glucose area under the curve of 75 g oral glucose tolerance test (r = 0.67 p < 0.01), fasting plasma insulin (r = 0.77 p < 0.001), serum total cholesterol (r = 0.71 p < 0.001) and serum triglyceride (r = 0.68 p < 0.01). From the present results, we speculate that excess visceral fat accumulation in myotonic dystrophy induces impaired glucose and lipid metabolism.

[A case of pontine hemorrhage showing hemiasomatognosia].

We reported a case of pontine hemorrhage showing hemiasomatognosia. A 74-year-old, right-handed man was admitted to our hospital complaining of numbness of left upper and lower extremities. Neurological examination revealed marked disturbance of superficial and deep sensation of left half of his body without hemiparesis and consciousness disturbance. Neuropsychologically, he had experience of having lost the perception of his left upper limb, which was referred to "conscious hemiasomatognosia" by Frederiks. Brain CT and MRI showed a pontine hematoma involving the right medial lemniscus, the right spinothalamic tract and the right medial longitudinal fasciculus. One week after the onset, hemiasomatognosia disappeared with improvement of sensory disturbance. These indicate that this form of hemiasomatognosia may be associated with transient blockage of somesthetic input.

[Alteration of atrial natriuretic peptide in progressive muscular dystrophy with congestive heart failure].

We measured plasma levels of atrial natriuretic peptide (ANP) in 9 patients of Duchenne muscular dystrophy (DMD) and 3 patients of Becker muscular dystrophy with congestive heart failure (CHF). Administration of digitalis, catecholamine and angiotensin converting enzyme inhibitor resulted in decrease of ANP levels as well as improvement of clinical symptoms of CHF and cardiomegaly. Four DMD patients whose ANP levels were more than 200 pg/ml after the treatment of CHF showed poor prognosis. These results suggest that ANP is a useful marker for the treatment of CHF in progressive muscular dystrophy.

[Electroglottographic evaluation of swallowing in Parkinson's disease].

The feasibility of using electroglottography (EGG) to record and measure aspects of swallowing was tested in 31 patients with Parkinson's disease (72.8 +/- 4.8 years) and 10 control subjects (74.6 +/- 4.4 years). The patients were divided into three groups: those feeling dysphagia frequently (D(+), n = 9), those feeling dysphagia occasionally (D(+/-), n = 8), and those not feeling dysphagia (D(-), n = 14). The instrument delivered a 250 microA, 52 kHz carrier signal to a pair of surface electrodes on each side of the larynx at the level of the thyroid lamina. Tissue impedance across the neck was recorded via EGG while the swallowing of 10 ml of water. Four trials were performed for each subject. Based on the analysis of EGG waveform, latency, number of phases and duration were measured. In the EEGs of D(+) group, the reproducibility of waveforms was poor, while the waveforms of D(-) group and control group showed higher reproducibility. The waveforms of D(+) group had significantly longer latency and more phases than those of D(-) and control groups. In D(+), the duration was significantly longer than that of control group. These results suggest that the EGG is a quantitative and noninvasive technique for analysis of swallowing in patients with Parkinson's disease.

[Postprandial hypotension in Parkinson's disease--the incidence and risk factor].

To clarify the incidence and risk factor of postprandial hypotension (PPH) in Parkinson's disease, a 75g oral glucose tolerance test (OGTT) was carried out in 23 patients (Hoehn-Yahr score II in 2, III in 18 and IV in 3) without postprandial symptoms. We defined the patients whose systolic blood pressure fell more than 20 mmHg during the 75g OGTT as group I and less than 20 mmHg as group II. In 14 patients with Parkinson's disease (61%), the systolic blood pressure fell more than 20 mmHg without symptoms. There were no significant differences in in age, disease duration, clinical stage or antiparkinsonian drug between groups I and II. However, the baseline systolic and diastolic blood pressures were significantly higher in group I than in group II. In group I patients, the maximum decrease in systolic blood pressure was seen after more than 60 minutes and serum insulin response was higher than in group II patients from 60 to 120 min. Systolic blood pressure declined accompanied by the rise of insulin. These results suggest that PPH occurs at high incidence in patients with moderately severe Parkinson's disease, and that high baseline blood pressure and abnormally high insulin response to glucose load may play a role, while medications for Parkinson's disease may not.

[Electroglottographic studies in myasthenia gravis patients--swallowing in exacerbation and remission stage].

We quantitatively evaluated the electroglottographic changes in swallowing in patients with generalized myasthenia gravis during exacerbation and remission stages. Five patients with myasthenia gravis (Osserman's stage IIB, 55.3 +/- 3.1 years old) and 6 healthy control subjects (55.3 +/- 1.1 years old) were examined. The instrument delivered a 450 microA. 52 kHz carrier signal to a pair of surface electrodes on each side of the larynx at the level of the thyroid lamina. Tissue impedance across the neck was recorded via electroglottography (EGG) while swallowing 10 ml of water. Four trials were performed for each subject, and the EGG waveform was analyzed. The EGG waveform during the exacerbation stage exhibited a more significantly increased number of phases and longer duration than that during remission. In myasthenia gravis patients, the number of phases and the duration from exacerbation to remission was more significantly reduced than those in controls at an interval of 3 years (p < 0.001, p < 0.02). These findings suggest that changes in the number of phases and duration of the EGG waveform are objective and quantitative parameters for measuring therapeutic effectiveness in treating swallowing disturbance due to myasthenia gravis. EGG is a useful and noninvasive technique for evaluating swallowing change in myasthenia gravis patents.

[A case of Kallmann syndrome with empty sella and arachnoid cyst].

We report a 28-year-old man of Kallmann syndrome with arachnoid cyst and empty sella. At age 22, he was admitted with acute slipped capital epiphysis and diagnosed as primary hypogonadotropinemia, because of no response to LH-RH before and after 7-day LH-RH injection. He was treated with androgen for only one year. On his second admission due to femoral head necrosis at age 28, the endocrinological evaluation suggested hypothalamic hypogonadotropinemia. Although he had mild hyposomia, we diagnosed him as Kallmann syndrome, because abnormalities of rhinencephalon was present on MRI. Arachnoid cyst in the middle cranial fossa and empty sella were also observed on MRI and the ballooning of the sella had been advanced on plain X-ray for these 6 years. As Kallmann syndrome is known to be accompanied with midline craniofacial anomalies, the dysplasia of sellar diaphragm might be originated by the same pathogenesis. In this case, empty sella might be caused by impaired CSF dynamics due to arachnoid cyst as well as possible constitutional anomaly of the diaphragm.

[Excess caloric intake induced severe hypercapnia in a patient with Duchenne muscular dystrophy on noninvansive positive pressure ventilation].

In many patients with neuromuscular diseases, respiratory failure is mainly caused by alveolar hypoventilation in their terminal stages. Malnutrition is one of the common and serious problems in patients with chronic respiratory failure. Energy consumption for breathing is remarkably high in respiratory compromised patients, causing subsequent increase of total energy expenditure. However, most patients have limited capacity of oral intake. Nutritional depletion is associated with wasting of respiratory muscles, impairment of respiratory drive, alteration of respiratory pattern, and pathological change of pulmonary parenchyma. These indicate that nutritional and ventilatory support is very important in these patients. However, overfeeding also may have detrimental influence on respiratory failure. We experienced a Duchenne muscular dystrophy (DMD) patient on noninvasive positive pressure ventilation (NIPPV) who developed hypercapnia after total parenteral nutrition (TPN). Analysis of clinical course of this patient revealed that there is a significant correlation between PaCO2 and caloric intake. Excess carbohydrate intake can precipitate fat synthesis which induces over-production of carbon dioxide (CO2). Since NIPPV doesn't have a closed circuit, there are some difficulties in respiratory management, such as air leakage to stomach and mouth, and airway obstruction. Failure to optimize NIPPV setting against increased CO2 load might cause hypercapnia in this patient. These suggest that evaluation of energy expenditure and design of nutritional program are essential to avoid hypercapnia due to nutritional support.

[Effective milrinone therapy to a Duchenne muscular dystrophy patient with advanced congestive heart failure].

We experienced a Duchenne muscular dystrophy (DMD) patient with severe congestive heart failure (CHF) successfully treated with milrinone. He had been diagnosed as having CHF since 24 years of age when he began to have mechanical ventilation with a nasal mask at home. Although angiotensin converting enzyme (ACE) inhibitor was effective for his CHF, cardiac function worsened year by year. Respiratory infection triggered the exacerbation of CHF at the end of 1997 (27 years old). On admission to our hospital on January 7, 1998, PaO2 was 48 mmHg and cardiothoracic ratio (CTR) was 62%. Both ventricles were dilated and ventricular wall motility was markedly reduced on ultrasonocardiography. Ejection fraction of the left ventricle (LVEF) was 5%. Serum brain natriuretic peptide (BNP) was 760 pg/ml. Continuous intravenous infusion of milrinone was started on January 8 at the rate of 0.25-0.35 microgram/kg/min. His general condition improved and LVEF increased up to 15% on January 27. No serious side effects were observed. Even after milrinone withdrawal, his cardiac condition remained stable until the end of February 1998. Temporary deteriorated CHF due to urinary tract infection was successfully treated by chemotherapy and milrinone. Subsequently he was discharged on March 13 and could stay in his home for 7 weeks uneventfully with milrinone infusion therapy. When he was readmitted to the hospital for evaluation of CHF on April 30, CTR was 44%, LVEF was 20% and BNP was 44 pg/ml. CHF is one of the life threatening complications for DMD. Although catecholamine is a well utilized agent for advanced CHF, it has limited effect in DMD, because beta receptors are down-regulated due to long-lasting cardiac dysfunction. Increased heart rate and arrhytmia are also serious problems during catecholamine therapy. Milrinone is a type III phosphodiesterase inhibitor having inotropic and vasodilatic actions with modest increase of heart rate and little torelance. Milrinone is probably effective in improving CHF of DMD and has less side effects as compared to catecholamine. We concluded that milrinone might improve quality of lives of DMD patients with advanced CHF, although further cumultative studies are necessary to confirm its effectiveness and safety.

[Electrolyte abnormalities and metabolic acidosis in two Duchenne muscular dystrophy patients with advanced congestive heart failure].

We experienced two Duchenne muscular dystrophy patients with advanced congestive heart failure, who showed abrupt severe hyponatremia, hyperkalemia and metabolic acidosis. Two patients received respiratory management, parenteral nutrition, and drugs including angiotensin converting enzyme inhibitors (ACEI). The patient 1 who was 19 years old showed abdominal pain, hematuria, diarrhea and disorientation. Laboratory findings were as follows; Na 120 mEq/L, K 7.3 mEq/L, BUN > 140 mg/dl (scale over), ACTH 20.2 pg/ml, cortisol 25 micrograms/dl, renin 40.7 ng/ml/hr and aldosterone 203 ng/dl. Arterial blood gas analysis (ABG) showed metabolic acidosis (pH 7.232). Combination therapy with hydrocortisone, glucose-insulin therapy (GIT) and NaHCO3 successfully rescued this patient. The patient 2 (28 years of age) was admitted to our hospital because of congestive heart failure. Laboratory findings were as follows; Na 129 mEq/L, K 5.5 mEq/L, BUN 60 mg/dl, cortisol 21 micrograms/dl, renin 36 ng/ml/hr and aldosterone 47 ng/dl. He complained abdominal discomforts from the next day of admission. Ten days after the admission Na, K and BUN were 111 mEq/L, 6.2 mEq/L and 154 mg/dl, respectively. ABG showed compensated metabolic acidosis. He fell into shock during GIT therapy. Laboratory findings at that time were as follows; Na 108 mEq/L, K 3.2 mEq/L, ACTH 77.6 pg/ml, cortisol 24 micrograms/dl, renin 58 ng/ml/hr and aldosterone 24 ng/dl. Although hydrocortisone was introduced, he could not recover and died. There are some reports about life-threatening electrolyte abnormalities and metabolic acidosis in the patients receiving ACEI. These phenomena were more frequent in patients with renal dysfunction and/or congestive heart failure. Hyponatremia, hypovolemia, combination therapy with nonsteroidal anti-inflammatory drugs (NSAID) and/or potassium sparing diuretics were reported as risk factors. We could not prove the correlation between the acute changes in our cases and ACEI. However ACEI is suspicious, because many of these risk factors were observed in our cases. Aldosterone was extremely elevated in the patient 1 when potassium was severely elevated. On the other hand, the patient 2 showed lower aldosterone level after correction of potassium than that on admission. Potassium is regarded as a major secretion factor of aldosterone for patients receiving ACEI. The fact the patient 2 fell into shock during GIT, tells us that we should use steroid simultaneously when we try to correct potassium quickly in severe cases, because acute reduction of potassium may decrease aldosterone. Today, ACEI is a common drug for CHF, so we should pay attentions that ACEI could cause such acute changes. To prevent such acute changes, excessive restriction of water and sodium intake should be avoided. If possible, NSAID and potassium sparing diuretics also should be avoided. Steroid therapy must be introduced rapidly when needed.

[A pilot study to establish a support system using SpO2 monitoring system and videoconference system for patients under home mechanical ventilation].

Development of portable ventilators has enabled patients suffering from respiratory failure to live outside hospitals. The number of patients with neuromuscular disorders receiving home mechanical ventilation (HMV) has been increasing year by year. However, Japanese social support services are not sufficient for these patients. Thus, we tried to establish a system to support patients under HMV using SpO2 monitoring system and videoconference system. Pulse oximeter was connected to a portable personal computer, which stores real-time data and send daily data to hospital. The computer in our hospital receives patient's data and prints out it as a trend-graph, which enables medical staffs to know patient's condition. When SpO2 deteriorates significantly, alarm system works automatically to warn patient's family. In case SpO2 worsens more severely, alarm also warns medical staffs in our hospital to call the patient to confirm patient's condition and to give advices. Videoconference system was also introduced to communicate quickly and correctly. Thirty-four patients with progressive muscular dystrophy attended this study after informed consents. They used these systems in the period between discharge and first visit to hospital. Videoconference system was utilized in only two patients, because it required a digital line. These systems were effective in decreasing anxieties of patients and their families about HMV. According to daily check, 16 of 34 patients showed temporary deterioration of respiratory condition, although all patients had been in good condition in hospital. Loosening of the belt of nasal mask, air leak from opened mouth were frequent causes for the deteriorations. These problems could be corrected smoothly after discussion and advices. Thus this system was very useful to establish good respiratory management in a short period. Monitoring system also detected troubles of ventilators. Two patients showed progressive worsening of SpO2 day by day, however we could not find any problems in their respiratory managements. When we checked the ventilator, there were lumps of dust at the upper-pressure limit valves, which caused air leak. It was revealed that 3 of 27 ventilators had the same troubles, which caused leaks more than 10% of the flow volume. This fact proved that this system was also effective to prevent severe troubles from unexpected problems about HMV. Videoconference system enabled us to communicate quickly and correctly. It was also convenient to give advices. It is quite difficult for ordinary people to explain and understand physical condition and methods to handle respirator with only oral communication. Visual communication solved these troubles almost completely. Although there are some problems about this system including high cost and quality of picture, it is undoubtedly powerful tool. It is natural for patients with respiratory failure to wish to stay their home, when they possessed portable respirators. So it is important to decrease the risk related to HMV. We verified that the combination of monitoring system and multimedia can produce effective support system. It is important to improve these systems and cut down the cost for popularization.

[Relationship between weight loss and dysphagia in patients with Parkinson's disease].

The purpose of this study was to investigate the relationship between weight loss and dysphagia in Parkinson's disease. We compared the height, body weight and the data of self-administered questionnaires concerning food intake and deglutition feelings in patients suffering from Parkinson's disease with normal controls. A structured interview was performed by nutritionists and nutrient intakes were calculated from the reported food intake over 5 days. Biochemical parameters were chosen from the chart. The subjects were 105 patients with Parkinson's disease, 34 males with a mean age of 67.7 +/- 8.6 years and 71 females with a mean age of 69.1 +/- 10.0 years (Hoehn-Yahr stage I6, II25, III51, IV20, V3). In addition, 47 family members were used as control subjects: 26 males, 70.6 +/- 7.6 years and 21 females, 64.9 +/- 7.7 years. Body mass index (BMI) in females with Parkinson's disease (20.2 +/- 3.5 kg/m2) was significantly lower (p < 0.005) than that in control females (23.0 +/- 3.0 kg/m2). There was no significant difference in BMI in males. The BMI was 21.9 +/- 3.0 kg/m2 in male patients with Parkinson's disease and 22.6 +/- 3.1 kg/m2 in controls. The occurrences of symptoms such as choking, cough, sputum, food in sputum, wet voice and pharyngeal discomfort following food intake in patients with Parkinson's disease vs. those in controls were 22% vs. 6%, 16% vs. 2%, 7% vs. 4%, 2% vs. 0%, 5% vs. 2% and 11% vs. 0%, respectively. Concerning symptoms such as choking, cough and pharyngeal discomfort, the occurrence was significantly more frequent in patients with Parkinson's disease than in controls (p < 0.05, p < 0.05, p < 0.05). We defined the dysphagic Parkinson patients as those who have at least one symptom of dysphagia such as choking, cough, sputum, food in sputum, wet voice and pharyngeal discomfort following food intake. The dysphagic subjects were present in 31% of Parkinson patients and in 7% of control subjects (p < 0.005), although half of the dysphagic Parkinson patients did not recognize it. No relationship between the occurrence of dysphagic symptoms and the Hoehn-Yahr stage was found. In patients with Parkinson's disease. BMI in the dysphagic group (19.1 +/- 3.6 kg/m2) was significantly lower than that in the non-dysphagic group (21.6 +/- 3.0 kg/m2) (p < 0.005). There was no relationship between BMI and the dose of levodopa. Patients in the dysphagic group showed significantly lower carbohydrate intake (186 +/- 49 g) than those in the non-dysphagic group (215 +/- 52 g) (p < 0.05). Biochemical nutritional parameters were lower in the dysphagic group than those in the non-dysphagic group; 6.6 +/- 0.7 g/dl vs. 6.9 +/- 0.4 g/dl (p < 0.005) in serum total protein, 3.8 +/- 0.5 g/dl vs. 4.1 +/- 0.4 g/dl (p < 0.01) in albumin and 173.4 +/- 33.0 mg/dl vs. 199.7 +/- 40.7 mg/dl (p < 0.05) in total cholesterol. These findings suggest that dysphagia, especially unrecognized dysphagia, plays a role in weight loss in Parkinson's disease.

[A case of myotonic dystrophy with intestinal pseudo-obstruction syndrome].

We experienced a patient of myotonic dystrophy presenting with intestinal pseudo-obstruction syndrome (IPOS). He was admitted to our hospital because of repetitive vomiting lasting for 24 hours. Abdominal CT revealed dilated stomach, duodenum and jejunum with extensive fluid residues. This patient was successfully treated with conservative therapy by using intravenous administration of metoclopramide. The etiology of IPOS in myotonic dystrophy is not well understood. In addition to normal findings of autonomic function tests such as R-R interval, Schellong test and sympathetic skin response, the efficacy of metoclopramide in treating IPOS suggests that acetylcholine (ACh) release is relatively preserved from the intestinal nerve plexus. Denervation hypersensitivity at the ACh receptors from long-standing dysfunction of autonomic system may be related to the cause of IPOS in myotonic dystrophy.

[Carvedilol effectiveness for left ventricular-insufficient patients with Duchenne muscular dystrophy].

To evaluate the efficacy of carvedilol for left ventricular dysfunction in Duchenne muscular dystrophy (DMD), we enrolled 8 patients with DMD who had elevated plasma atrial natriuretic peptide (ANP) or brain natriuretic peptide (BNP), and a low ejection fraction (EF < 40%) in echocardiography. Written informed consent was obtained from all of them. Four agreed to be treated with oral carvedilol 0.3125-1.25 mg/day (10.1-40.3 micrograms/kg/day) for 6 months (treated group). The others served as the controls (untreated group). In both groups, we evaluated clinical symptoms, plasma ANP, BNP and EF before, 3 and 6 months after the trial, and iodine-123 metaiodobenzylguanidine (123I-MIBG) imaging at 6 month interval. Parameters in the treated and untreated groups before respectively were ANP, 83.8 +/- 17.5 and 89.5 +/- 44.4 pg/ml (mean +/- SEM); BNP, 169.0 +/- 46.2 and 186.3 +/- 61.8 pg/ml; EF, 24.0 +/- 2.2 and 16.5 +/- 1.9%; Heart/Mediastrinum ratio of the 123I-MIBG delayed image, 1.65 +/- 0.08 and 1.6 +/- 0.10; and Washout rate, 46.5 +/- 8.6 and 41.4 +/- 7.8. These values did not change significantly before and 6 months after for either group. Clinical symptoms also did not change in either group. Carvedilol therapy did not change the left ventricular dysfunction in DMD.