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.

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.

Cortical mapping of gait in humans: a near-infrared spectroscopic topography study.

While we have a fair understanding of how and where forelimb-hand manipulative movements are controlled by the neocortex, due to functional imaging studies, we know little about the control of bipedal movements such as walking because of technical difficulties. We succeeded in visualizing cortical activation patterns of human gait by measuring relative changes in local hemoglobin oxygenation using a recently developed near-infrared spectroscopic (NIRS) topography technique. Walking activities were bilaterally associated with increased levels of oxygenated and total hemoglobin in the medial primary sensorimotor cortices and the supplementary motor areas. Alternating foot movements activated similar but less broad regions. Gait imagery increased activities caudally located in the supplementary motor areas. These findings provide new insight into cortical control of human locomotion. NIRS topography might be also useful for evaluating cerebral activation patterns during pathological gait and rehabilitative intervention.

Coagulation and fibrinolysis disorder in muscular dystrophy.

To investigate whether there are any basic abnormalities of coagulation and fibrinolysis in muscular dystrophy, we measured serum levels of the MM isozyme of creatine kinase (CK-MM), fibrin and fibrinogen degradation products (FDP), plasma levels of fibrinogen, antithrombin (AT), and D-dimer in 36 patients with Duchenne muscular dystrophy (DMD), 11 with Becker muscular dystrophy (BMD), 5 with Fukuyama congenital muscular dystrophy (FCMD), 5 with myotonic dystrophy (MyD), and 5 with spinal muscular atrophy (SMA) type 2. FDP levels were elevated in the patients with DMD, BMD, and FCMD (1.0 to 84.9 microg/ml), but not in the patients with MyD and SMA type 2. In DMD, BMD, and FCMD, FDP levels significantly correlated with CK-MM, but not with age, fibrinogen, AT, D-dimer, and type of dystrophy (multiple regression analysis; r(2) = 0.814, P < 0.0001). These findings suggested that enhanced coagulation and fibrinolysis are associated with muscle degeneration in patients with DMD, BMD, and FCMD.

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.

[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.

[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.

Treadmill training with body weight support: its effect on Parkinson's disease.

OBJECTIVE: To test whether body weight-supported treadmill training (BWSTT) is effective in improving functional outcome of patients with Parkinson's disease. DESIGN: Prospective crossover trial. Patients were randomized to receive either a 4-week program of BWSTT with up to 20% of their body weight supported followed by 4 weeks of conventional physical therapy (PT), or the same treatments in the opposite order. Medications for parkinsonism were not modified throughout the study. SETTING: Inpatient rehabilitation unit for neurologic diseases. SUBJECTS: Ten patients (5 men, 5 women) with Hoehn and Yahr stage 2.5 or 3 parkinsonism; mean age 67.6 years, mean duration of Parkinson's disease 4.2 years. MAIN OUTCOME MEASURES: The Unified Parkinson's Disease Rating Scale (UPDRS), ambulation endurance and speed (sec/10 m), and number of steps for 10-meter walk. RESULTS: The mean total UPDRS before/after BWSTT was 31.6/25.6, and before/after PT was 29.1/28.0. Analysis of covariance for improvement of UPDRS demonstrated a significant effect of type of therapy (F(1, 16) = 42.779, p < .0001) but not order of therapy (F(1, 16) = 0.157, p = .697 1). Patients also had significantly greater improvement with BWSTT than with PT in ambulation speed (BWSTT, before/after = 10.0/8.3; PT, 9.5/8.9), and number of steps (BWSTT, 22.3/19.6; PT, 21.5/20.8). CONCLUSIONS: In persons with Parkinson's disease, treadmill training with body weight support produces greater improvement in activities of daily living, motor performance, and ambulation than does physical therapy.

Improved functional outcome in patients with hemorrhagic stroke in putamen and thalamus compared with those with stroke restricted to the putamen or thalamus.

BACKGROUND AND PURPOSE: We analyzed the effect of late intensive inpatient rehabilitation on the functional outcome of patients with subcortical hemorrhagic stroke. METHODS: Patients who were nonambulatory with hemorrhagic stroke in the internal capsule and putamen (n=55), the thalamus (n=24), or all 3 regions (n=15) underwent intensive inpatient rehabilitation. Patients with surgical intervention or an episode of ventricular hemorrhage were excluded. Lesion location was evaluated by MRI 4 months after the ictus. RESULTS: Demographic data, initial disability, and impairment measures were comparable in the 3 groups. Functional outcome demonstrated significant differences in mobility subscores (P<0.05) of the Functional Independence Measure such that patients with injury in the 3 regions were more likely to ambulate independently than were patients in the other groups. Lesion location data demonstrated that the ventral anterior nucleus of the thalamus was always spared; the ventral posterior (lateral and medial) nucleus was always damaged, and the ventral lateral nucleus was frequently damaged. Putaminal damage always included the postcommissural area. In addition, the entire posterior half limb of the internal capsule was always damaged. CONCLUSIONS: Subcortical lesions to multiple structures in the basal ganglia-thalamocortical motor circuits permitted enhanced motor recovery. Lesion location predicted the level of independent ambulation and the rate of recovery in patients with stroke who were nonambulatory before neurorehabilitation therapy.

[MRSA infection control in the wards for progressive muscular dystrophy: the effects of encouraged handwashing].

There are currently 27 national hospitals with the wards for progressive muscular dystrophy (PMD) in Japan. Today, most patients in these wards are severely motor handicapped and complicated with respiratory and/or cardiac failure. Malnutrition, dysphagia, insufficient respiratory tract clearance are common problems that cause fragility to infection. Although progress in the treatment of infection have remarkably prolonged their life-span, frequent use of antibiotics is a major factor for occurrence of drug resistant bacteria. Since we had the first case of methicillin resistant Staphylococcus aureus (MRSA) infection in 1994 in our hospital, the number of MRSA carriers increased year by year. To prevent the spread of MRSA, we revised our infection precaution manual and surveyed its consequence. We encouraged handwashing of staffs, introduced green tea in cleansing genital region and abolished the use of a private room except a patient with severe seborrheic eczema. The revision of the manual resulted in decrease of MRSA carriers. The surveillance revealed that many patients had MRSA in genital regions, although there were no relationships between colonization of MRSA and severlity of disability or complications. It was strongly suggested that toilettary care by the hands of nursing staffs was a major factor of transferring MRSA. Our study revealed that encouragement of handwashing is more powerful in preventing the spreading MRSA, and more favorable for quality of lives of PMD patients than isolating the patients.

[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.

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.

[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.

Middle cerebral artery stroke that includes the premotor cortex reduces mobility outcome.

BACKGROUND AND PURPOSE: The premotor cortex (PMC) (Brodmann 6) contributes uniquely to proximal upper and lower limb power and plays a role in the organization of motor behaviors. We assessed the degree to which PMC damage affected functional outcome. METHODS: We prospectively compared the functional outcome of patients with a first stroke in the middle cerebral artery distribution that either left the PMC intact (PMC-; n=19) or damaged the PMC (PMC+; n=12). The Functional Independence Measure for disability and the motor score of the Stroke Impairment Assessment Set for impairment assessed outcome. RESULTS: Demographic and clinical features and lesion volume were comparable for the PMC+ and PMC- groups. However, the PMC- group demonstrated significant gain in mobility and in proximal leg movement. This focal improvement contributed to the trend in the PMC- group toward greater independent ambulation. CONCLUSIONS: Decreased motor recovery of proximal lower limbs in humans with PMC damage supports the idea that it is the origin of corticoreticulospinal pathways that subserve proximal lower extremity function. Furthermore, persistent proximal weakness after PMC damage may amplify other motor impairments, which include defects in planning, initiating, and sequencing. Neurorehabilitation outcomes may contribute to a more detailed functional anatomy after stroke and partial recovery.

[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.

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.

[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.

[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.