Guillain-Barré Syndrome (GBS) is an acute monophasic immune-mediated neuropathy, generally considered to be of good prognosis. However, 15–20% of GBS patients cannot walk independently at six months from onset. Poor prognostic factors for long-term functional disability included old age, preceding diarrhea, muscle weakness on admission and on day 7 from admission, severe GBS disability score at two weeks from admission and IgG antibody against GD1a/GD1b ganglioside complex. Factors related with requirement of mechanical ventilation included the time from onset to admission <7 days, muscle weakness on admission, facial and/or bulbar weakness and IgG antibody against GQ1b. Recently modified Erasmus GBS outcome score (mEGOS) and Erasmus GBS respiratory insufficiency score (EGRIS) were reported as prognostic factors for the long-term functional disability and respiratory insufficiency. Those were designed on Dutch patients. The usefulness of these tools in Japan or other countries remained unknown. The authors validated mEGOS and EGRIS on Japanese GBS patients in Japanese GBS outcome study, which revealed that these tools were also adaptable on Japanese GBS patients. To identify clinical and biological factors of GBS in more detail, such a large scale prospective study as International GBS outcome study (IGOS) is warranted.
We have developed a partial peptide of RANKL (receptor activator of NF-κB ligand) that suppresses TLR (toll-like receptor)-related inflammation via RANKL/RANK (receptor activator of nuclear factor-κB) signals in the acute phase of ischemic stroke. This peptide has been found to be a therapeutic agent for ischemic stroke that can be used in combination with tPA in a mouse model. Based on the findings, we are working on translational research to aim for clinical application of this peptide through collaboration with pharmacy companies. However, the problem is that the need for development of medication in the acute stage of ischemic stroke is currently low in pharmaceutical companies due to the failure of many investigational drugs in the past. To overcome the problem, we are examining the effects of this peptide in other diseases included in the company’s priority areas and explaining the environmental changes in the clinical trials due to the development of endovascular treatment in the acute stage of ischemic stroke.
The effect of malnutrition on intractable neurological diseases, including neurodegenerative diseases, is variable. Nutritional status is dependent on various factors such as disease characteristics; various symptoms including dysphagia, respiratory dysfunction, motor weakness, muscle rigidity, involuntary movement, and ataxia; and changes in energy metabolism caused by diseases and its stage. Nutritional therapy for patients with intractable neurological diseases requires the provision of tailor-made supports for individual patients based on sharing of knowledge and experience in multidisciplinary members. Evidence on nutritional management in the field of neurology is limited compared to that in the fields of surgery and internal medicine. This article thus aimed to describe the activities of the nutrition support team (NST) at our hospital that is specialized in intractable neurological diseases and the knowledge obtained through the activities.
A 71-year-old man presented with progressive muscle weakness of the four limbs in November 2014. His symptoms had started from the left leg in 2008, resulting in frequent falls. In 2011, he became unable to stand up without a handrail due to weakness of the both legs. Physical examination showed almost symmetric muscle weakness of the arms and legs; MMT4. The CK level was slightly elevated of 304 IU/l. The patient was diagnosed as having inclusion body myositis based on the muscle biopsy findings showing many fibers with rimmed vacuoles in addition to mononuclear cell infiltrating into the endomysium, surrounding and sometimes invading into non-necrotic muscle fibers. Anti-PM/Scl-75 antibodies were positive. Muscle strength improved after intravenous immunoglobulin therapy, although the effect was only temporary. This rare case suggests the autoimmunological etiology in inclusion body myositis.
We report a case of a 29-year-old woman with spinal muscular atrophy (SMA) type II who developed severe ketoacidosis after short-term starvation. She was hospitalized with lower respiratory tract infection. Although her symptoms improved after administration of intravenous antibiotic agents, her food intake gradually decreased. On the 7th day of hospitalization, she experienced abdominal pain followed by vomiting, after which she was unable to eat. Approximately 12 h later, she suffered from shock, accompanied with disturbance of consciousness, and she was admitted to the intensive care unit. She was diagnosed with ketoacidosis based on arterial blood gas analyses and urine test results. On receiving continuous infusion of glucose and insulin, her ketoacidosis was rapidly resolved and her symptoms completely recovered by the next day. To prevent the recurrence of ketoacidosis, we provided a diet plan based on indirect calorimetry results. However, ketoacidosis recurred twice, at 12 months and 16 months after discharge, both within 24 h of the onset of the fasting state. In addition to insufficient glycogen storage because of chronic malnutrition, poor gluconeogenesis or poor ketone body consumption due to skeletal muscle atrophy was believed to increase the risk of acute-onset, severe ketoacidosis after short-term starvation. Clinicians must note that patients with SMA are prone to ketoacidosis and that they must be promptly treated.
A 67-year-old woman who had undergone laparoscopic proximal gastrectomy for early gastric cancer 10 months previously was admitted to our hospital due to dysarthria. Brain MRI demonstrated acute multiple small infarcts in the right middle cerebral artery (MCA) and the right posterior inferior cerebellar artery (PICA) territory, and she was diagnosed as embolic stroke. Anticoagulant therapy did not prevent further ischemic stroke. No embolic sources were detected by MR angiography, carotid duplex sonography, transthoracic and transesophageal echocardiography, and Holter electrocardiography. We also performed upper gastrointestinal endoscopy and contrast-enhanced CT of the thoracoabdominal area, but there was no evidence of local recurrence or lymph node metastases of gastric cancer. As the ALP and D-dimer levels were gradually increasing, we performed PET/CT, which revealed fluorodeoxyglucose (FDG) uptake in the vertebra bone, and disseminated carcinomatosis of bone marrow with early gastric cancer was diagnosed after bone marrow biopsy on Day 41. After undergoing chemotherapy, she had no further stroke and died on Day 207.
Two men (Case 1, 74 years old; Case 2, 65 years old) developed cardioembolic stroke due to self-interruption of anticoagulants for treating atrial fibrillation. They both had mobile thrombus in the left atrial appendage. In Case 1, a left atrial thrombectomy was scheduled on day 8, but infarction re-occurred on the morning of the the surgery, producing neurological sequelae. In Case 2, left atrial thrombectomy and left atrial appendage closure were performed successfully on day 8. The indication and timing of cardiac thrombectomy after the onset of cerebral infarction have not been standardized, and they seem to differ among individuals. Therefore, in the future, the optimal timing of left atrial thrombectomy should be decided based on the size and morphology of the left atrial thrombus, the size of the cerebral infarction and the presence or absence of hemorrhagic infarction.
We herein report the case of a 54-year-old man who abused toluene for 25 years and gradually developed gait disturbance. Neurological findings showed mild cognitive impairment, hearing impairment, dysarthria, marked hyperreflexia of the limbs, spastic paraplegia, slight impairment of deep sensation, and urinary disturbance; however, there was no muscular atrophy. Serum antibodies against human T-lymphocytic virus 1 and aquaporin 4 were negative. Biochemical analysis did not show an increase in very-long-chain fatty acids. The cerebrospinal fluid was normal for the cell number and protein level but positive for oligoclonal IgG band, with a mildly increased IgG index. Brain MRI showed marked high intensity in the bilateral periventricular, deep cerebral and subcortical white matter as well as atrophy of the cerebrum, cerebellum and brainstem, and thinning of the corpus callosum. Spinal MRI showed marked atrophy of the lower cervical spinal cord, thoracic spinal cord, and conus medullaris. Spinal cord lesions are extremely rare in chronic toluene intoxications, and there are no reports of spinal cord atrophy. Lateral and ventral columns of the spinal cord are responsible for pyramidal tract signs, and insidious ongoing inflammation related to chronic toluene intoxication in the central nervous system is predicted to underlie the pathogenesis.
Door to groin puncture time is one of the determinants of clinical outcome in patients treated with endovascular thrombectomy (EVT). We have recently initiated a protocol, direct transfer to angiographic site, for patients transferred from outside hospitals. In this retrospective study, we investigated whether our new protocol had succeeded in shortening the door to groin puncture time. Data on consecutive patients with an occlusion at internal carotid artery or middle cerebral artery treated with EVT transferred from outside hospital between July 2012 and December 2018 were studied. Good outcome was defined as modified Rankin Scale score (mRS) ≤1 at 3 months. Forty (46%) patients were directly transferred to angiographic suite, 27 (19%) were indirectly transferred after CT, and 20 (23%) were after MRI. Onset to admission time was similar among the 3 groups (P = 0.711), while door to groin puncture time was significantly shorter in patients directly transferred to angiographic suite compared to those after CT as well as MRI (median 22 [25%–75%, 16–31] minutes vs. 31 [27–40], vs. 84 [58–124], P < 0.001). The rates of reperfusion with ≥ Thrombolysis in Cerebral Infarction 2b were similar among the 3 groups (88% vs. 85% vs. 90%, P = 0.886). The incidences of symptomatic intracerebral hemorrhage were also similar as 8% vs. 4% vs. 5% (P = 0.796). At 3 months after stroke, 16 (40%) patients in the 11 (41%) in those after CT, and 9 (45%) in those after MRI had the good outcome (P = 0.931). Direct transfer to angiography suite can shorten the onset to groin puncture time with safety.