[A Questionnaire Survey of Nurses on Pharmacist Services in the Emergency Rooms at Tokushukai Medical Group].

We conducted a multicenter survey of emergency room nurses to obtain information that would be useful for the establishment of pharmacist services in emergency rooms. Notably, 199 valid responses were obtained from 12 hospitals. The most common expectation from pharmacists in the emergency room was "drug management" (70.9%), followed by "providing information to physicians regarding the patient's medication history" (59.3%), and "auditing of dosage and interaction" (57.3%). The working arrangements that the survey respondents wanted regarding pharmacists in emergency rooms were: 24 h pharmacist (41.7% wanted this arrangement), day-shift pharmacist (24.6% wanted this arrangement), 24 h on-call (17.1% wanted this arrangement), day-shift on-call (5.0% wanted this arrangement), telephone support (11.1% wanted this arrangement), and 0.5% said that there was no need for pharmacists. In the analysis of factors affecting nurse satisfaction, day-shift pharmacist was a significant factor. We hope that the results of this survey will be used as a guide for the development of emergency room pharmacist services tailored to the unique characteristics and actual working conditions of each hospital.

X-linked Myotubular Myopathy Manifesting Carrier with Central and Peripheral Nervous System Involvement.

X-linked myotubular myopathy (XLMTM) is a rare genetic disorder caused by X-linked mutations in the MTM1 gene. Although heterozygous females are typically asymptomatic, affected cases have recently been reported. We herein report a case of XLMTM manifesting carrier of the pathogenic c.206dupG mutation in MTM1 with uncommon extramuscular symptoms. She developed gaze nystagmus and cognitive impairment in addition to muscle weakness. Electrophysiological studies and brain magnetic resonance imaging indicated the involvement of the central and peripheral nervous systems. XLMTM manifesting carriers may have a wider spectrum of clinical phenotypes than currently assumed. Appropriate follow-up of extramuscular and conventional muscular manifestations is important in such cases.

Association of Pars Defect Type with Clinical Outcome after Smiley Face Rod Methods for Terminal-Stage Spondylolysis.

Introduction: The smiley face rod method is an effective treatment for symptomatic terminal-stage spondylolysis. However, the risk factors for treatment failure are unknown. We investigated the association of pars defect type with the treatment outcomes of this method. Methods: We retrospectively examined data from 34 patients (18.0±6.7 years) with terminal-stage spondylolysis who underwent surgery using the smiley face rod method. The mean follow-up period was 44.9±21.4 months. The patients were divided into 2 groups: pars defect without bone atrophy or sclerosis (group A; 18 patients), and with bone atrophy and sclerosis (group B; 16 patients). We evaluated and compared the visual analog scale (VAS) score for back pain, bone union rate, and time to return to preinjury athletics level between the groups. Fisher exact and paired t tests were used to compare the variables between groups. The VAS score between the groups was compared using a 2-factor repeated-measures analysis of variance. Results: Within groups, the VAS score was significantly different over time (p<0.001). The VAS scores between groups were not significantly different. Patients in group A had a significantly higher bone union rate per pars at 6 months (group A, 65.7%; and group B, 37.5%, p=0.028) and 24 months after surgery (group A, 97.1%; and group B, 75.0%, p=0.011). All patients returned to their respective sports, and no significant differences were observed in the time to return to preinjury athletics level between the groups (p=0.055). Conclusions: The type of pars defect are associated with bone union after the smiley face rod method, but have little effect on postoperative symptoms.

Erratum for A Novel Technique for Pars Defect Direct Repair with a Modified Smiley Face Rod for Spondylolysis and Isthmic Spondylolisthesis.

[This corrects the article DOI: 10.22603/ssrr.2023-0021.].

A Novel Technique for Pars Defect Direct Repair with a Modified Smiley Face Rod for Spondylolysis and Isthmic Spondylolisthesis.

Introduction: Lumbar spondylolysis is a common fatigue fracture of the pars interarticularis of the lamina of the lumbar spine in adolescent athletes presenting with pars clefts. Some pseudarthrotic lumbar spondylolysis causes low back pain or radiculopathy. This study presents a case of pseudarthrotic lumbar spondylolysis that was successfully treated using a modified smiley face rod technique. Technical Note: We developed a modified smiley face rod technique, which places pedicle screws in the lateral edge of the pedicle to preserve the erector spinae muscles and inserts a U-shaped rod between the spinous processes to preserve the supraspinous ligament. When a U-shaped rod penetrates the interspinous ligament subcutaneously, the resection of the supraspinous ligaments can be avoided. When the screw head is positioned more anterolaterally, a compression force is applied perpendicular to the surface of the pars cleft by rod clamping. This intrasegmental fusion technique preserves the mobile segment and simultaneously repairs the pars cleft. It is less invasive and more appropriate than interbody fusion for young athletes to avoid the possibility of future adjacent segment disorders. Conclusions: This is a minimally invasive procedure that can easily achieve bone fusion and should be introduced for patients who are suffering from the symptoms of pseudarthrotic lumbar spondylolysis.

Sacroiliac Joint Ankylosis Decreases Intervertebral Fusion Rate in L5/S1 Single Intervertebral Transforaminal Lumbar Interbody Fusion.

Background Transforaminal lumbar interbody fusion (TLIF) is a common surgical procedure for lumbar spondylolisthesis and intervertebral foraminal stenosis. Sacroiliac joint ankylosis is also known to occur in patients without axial spondyloarthritis. When sacroiliac joint bony ankylosis occurs and sacroiliac joint mobility is lost, stresses from the lower extremities to the lumbar spine are no longer buffered and are expected to be concentrated between the fifth lumbar (L5) and the first sacral (S1) vertebrae. We hypothesized that sacroiliac joint bony ankylosis could adversely affect L5/S1 intervertebral fusion and investigated the postoperative intervertebral fusion rate in single intervertebral TLIF on L5/S1 among patients with bony ankylosis of the sacroiliac joint. Methods Seventy-two patients who had undergone TLIF in the L5/S1 single intervertebral segment since 2014 and had a follow-up of at least one year after surgery were included in the study. Seventy-two patients were divided into the following two groups for comparison: group A consisted of 17 patients with bony ankylosis of the sacroiliac joint on either side on preoperative CT, and group N consisted of 55 patients without ankylosis. We investigated the intervertebral segment fusion rate one year postoperatively. Fisher's exact tests were used for statistical analysis, with a significance level of P < 0.05. Results Twelve patients (71%) in group A and 50 patients (91%) in group N had a fusion of the L5/S1 intervertebral segment one year after TLIF surgery, with a significantly lower rate in group A (P = 0.049). Conclusions We conclude that the presence of preoperative sacroiliac joint bony ankylosis is a risk factor for postoperative intervertebral fusion failure after single-segment TLIF at L5/S1.

Anterior interosseous nerve syndrome following infection with COVID-19: a case report.

BACKGROUND: Various neurological manifestations associated with coronavirus disease 2019 have been increasingly reported. Herein, we report a rare case of anterior interosseous nerve syndrome, which occurred 5 days after the onset of coronavirus disease 2019. CASE PRESENTATION: A 62-year-old Asian woman with a history of coronavirus disease 2019 who developed a complete motor deficit in the left flexor pollicis longus and pronator quadratus without sensory deficits. The symptoms appeared as a sudden onset fatigue and severe pain of the left arm, 5 days after the onset of coronavirus disease 2019. She noticed paralysis of the left thumb at 2 weeks after the onset of coronavirus disease 2019. Electromyography assessment of the anterior interosseous nerve-dominated muscles revealed neurogenic changes such as positive sharp wave and fibrillation in flexor pollicis longus and pronator quadratus, confirming the diagnosis of anterior interosseous nerve syndrome. There were no other diseases that could have resulted in peripheral nerve palsy. We performed a functional reconstruction surgery of the thumb by tendon transfer from the extensor carpi radialis longus to the flexor pollicis longus. The patient reported a good patient-reported outcome (2.27 points in QuickDASH Disability/Symptom scoring and 5 points in Hand20 scoring) at final follow-up (1 year after the surgery). CONCLUSION: This case highlights the need for vigilance regarding the possible development of anterior interosseous nerve syndrome in patients with coronavirus disease 2019. Tendon transfer from extensor carpi radialis longus to flexor pollicis longus can provide good functional recovery for unrecovered motor paralysis after anterior interosseous nerve syndrome.

Percutaneous pedicle screw fixation was effective for bone regeneration after a huge vertebral defect due to intractable pyogenic spondylitis caused by methicillin-resistant Staphylococcus aureus: a case report.

BACKGROUND: Pyogenic spondylitis by methicillin-resistant Staphylococcus aureus (MRSA) is known to be intractable. In the past, the insertion of an implant into infected vertebra was considered contraindicated in affected patients because it may exacerbate the infection, but there are increasing numbers of reports indicating the usefulness of posterior fixation to correct instability and alleviate infection. Bone grafting is often required to repair large bone defect due to infection, but free grafts can exacerbate infection and are controversial. CASE PRESENTATION: We present the case of a 58-year-old Asian man with intractable pyogenic spondylitis who had repeated septic shocks due to MRSA. Back pain from repeated pyogenic spondylitis caused by a huge bone defect in L1-2 rendered him unable to sit. Posterior fixation by percutaneous pedicle screws (PPSs) without bone transplantation improved spinal stability and regenerated bone in the huge vertebral defect. He regained his activities of daily living, had no reoccurrence of pyogenic spondylitis nor bacteremia, and was completely cured of the infection without antibiotics after removal of all screws. CONCLUSIONS: For intractable MRSA pyogenic spondylitis with instability accompanied by a huge bone defect, posterior fixation using PPSs and administration of antibacterial agents stopped the infection, allowed the bone to regenerate, and recovered the patient's activities of daily living.

Myopathic changes caused by protein aggregates in adult-onset spinal muscular atrophy.

Spinal muscular atrophy (SMA), an autosomal-recessive lower motor neuron disease, causes progressive proximal muscle waste and weakness. It remains unclear whether myopathic changes are involved in pathogenesis. We encountered a patient with adult-onset SMA caused by a homozygous deletion in exon 7 of the survival motor neuron 1 (SMN1) gene who had had four copies of SMN2 exon 7. Muscle biopsy showed neurogenic features of groups of atrophic fibers, fiber-type grouping, and pyknotic nuclear clumps associated with fibers with rimmed vacuoles. Immunohistochemistry revealed sarcoplasmic aggregates of phosphorylated TDP-43 and p62 but not SMN. This study demonstrated myopathic changes with the accumulation of phosphorylated p62 and TDP-43 in the muscles of a patient with SMA, suggesting that abnormal protein aggregation may be involved in myopathic pathology.

Autoimmune Polyglandular Syndrome with Refractory Gait Disturbance.

Autoimmune polyglandular syndrome (APS) causes autoimmune diseases of multiple organs and can also present with neurological symptoms. We here report a 58-year-old man who presented with progressive gait disturbance that had started 7 years ago. He had spasticity, reduced deep sensations, and truncal cerebellar ataxia. Laboratory examinations revealed autoantibody-related cobalamin deficiency and the presence of anti-thyroid antibodies and anti-glutamic acid decarboxylase antibodies. His gait worsened after cobalamin replenishment, but additional steroid therapy was effective. APS can cause refractory gait disturbance that requires not only cobalamin replenishment but also immunotherapy.

Posterior lumbar interbody fusion for dysplastic lumbar spondylolisthesis with high-grade slippage in two adolescent siblings: two case reports.

BACKGROUND: Lumbar spondylolisthesis is reported to present with a familiar pattern, with the dysplastic type of spondylolysis being minor but more hereditary than the isthmic type. Siblings presenting during adolescence with neurological symptoms owing to high-grade dysplastic-type spondylolisthesis are rare. CASE PRESENTATION: The older brother suffered from left leg pain and numbness and dysesthesia of the right posterior thigh and calf and could not walk without a crutch at the age of 15 years. He had canal stenosis with disc bulging and dysplastic bilateral facet joint at L5/S1. The L5 vertebral body was slipped anterior downward to S1, with a round-shaped S1 cranial endplate. We diagnosed dysplastic-type spondylolisthesis and performed posterior lumbar interbody fusion at L5/S with mild reduction and sublaminar wiring at L4/5. The younger brother had no neurological symptoms at age 14 years but suffered from bilateral lower leg numbness at age 18 years. He had canal stenosis with disc bulging at L4/5 and L5/S1 and dysplastic bilateral facet joint at L5/S1 with right pars defect. The L5 vertebral body was vertically displaced anterior to the S1 vertebral body, with an S1 round-shaped cranial endplate. We diagnosed dysplastic-type spondylolisthesis, and posterior lumbar interbody fusion at L4/5 and L5/S with reduction was performed. Their neurological symptoms of the lower legs disappeared, and interbody bone fusion was obtained. CONCLUSIONS: The surgical technique for high-grade dysplastic spondylolisthesis remains controversial in terms of in situ fusion versus reduction. We recommend that surgery be performed promptly at the end of bone maturation because neurological symptoms often appear at the end of bone maturation. Because high-grade slips are rare but siblings may be present, the sibling should also be screened when dysplastic spondylolisthesis is detected.

Postoperative Blood Loss Including Hidden Blood Loss in Early and Late Surgery Using Percutaneous Pedicle Screws for Traumatic Thoracolumbar Fracture.

INTRODUCTION: Some reports revealed that hidden blood loss (HBL) during surgery for traumatic thoracolumbar fracture cannot be ignored, even when using a percutaneous approach. Using percutaneous pedicle screws (PPS) for traumatic thoracolumbar fracture, this study aimed to compare estimate blood loss (EBL), including HBL, between early and late fixation. METHODS: This investigation was a retrospective study. In the present study, data from 39 patients who underwent posterior spinal stabilization using PPS for single-level thoracolumbar fracture have been included. We divided the patients into an early group (group E) (n=20) in whom surgery was conducted within 3 days of fracture and a late group (group L) (n=19) in whom surgery was conducted more than 3 days after fracture. We evaluated hemoglobin (Hb) on the day of injury, and 1, 3 or 4, and 7 days after surgery, EBL, HBL, and transfusion requirement. RESULTS: Hb on day 1 (group E: 12.2±1.7 g/dL, group L: 12.3±1.6 g/dL) was significantly less than that on the injured day (group E: 14.2±1.7 g/dL, group L: 13.9±1.7 g/dL) in both groups. The values of Hb and EBL were not significantly different at any time between the two groups. HBL (group E: 487±266 mL, group L: 386±305 mL) was not significantly different between the two groups. No patients required transfusion in either group. CONCLUSIONS: EBL in early fixation using PPS for traumatic thoracolumbar fracture is not significantly different compared with that in late surgery from days 1 to 7 postoperatively. Early fixation using PPS for traumatic thoracolumbar fracture does not result in negative outcomes any more than those in late surgery in terms of blood loss.

Randomized trial of granulocyte colony-stimulating factor for spinal cord injury.

Attenuation of the secondary injury of spinal cord injury (SCI) can suppress the spread of spinal cord tissue damage, possibly resulting in spinal cord sparing that can improve functional prognoses. Granulocyte colony-stimulating factor (G-CSF) is a haematological cytokine commonly used to treat neutropenia. Previous reports have shown that G-CSF promotes functional recovery in rodent models of SCI. Based on preclinical results, we conducted early phase clinical trials, showing safety/feasibility and suggestive efficacy. These lines of evidence demonstrate that G-CSF might have therapeutic benefits for acute SCI in humans. To confirm this efficacy and to obtain strong evidence for pharmaceutical approval of G-CSF therapy for SCI, we conducted a phase 3 clinical trial designed as a prospective, randomized, double-blinded and placebo-controlled comparative trial. The current trial included cervical SCI [severity of American Spinal Injury Association (ASIA) Impairment Scale (AIS) B or C] within 48 h after injury. Patients are randomly assigned to G-CSF and placebo groups. The G-CSF group was administered 400 µg/m2/day × 5 days of G-CSF in normal saline via intravenous infusion for five consecutive days. The placebo group was similarly administered a placebo. Allocation was concealed between blinded evaluators of efficacy/safety and those for laboratory data, as G-CSF markedly increases white blood cell counts that can reveal patient treatment. Efficacy and safety were evaluated by blinded observer. Our primary end point was changes in ASIA motor scores from baseline to 3 months after drug administration. Each group includes 44 patients (88 total patients). Our protocol was approved by the Pharmaceuticals and Medical Device Agency in Japan and this trial is funded by the Center for Clinical Trials, Japan Medical Association. There was no significant difference in the primary end point between the G-CSF and the placebo control groups. In contrast, one of the secondary end points showed that the ASIA motor score 6 months (P = 0.062) and 1 year (P = 0.073) after drug administration tend to be higher in the G-CSF group compared with the placebo control group. Moreover, in patients aged over 65 years old, motor recovery 6 months after drug administration showed a strong trend towards a better recovery in the G-CSF treated group (P = 0.056) compared with the control group. The present trial failed to show a significant effect of G-CSF in primary end point although the subanalyses of the present trial suggested potential G-CSF benefits for specific population.

[A case of inflammatory cerebral amyloid angiopathy with white matter lesions appearing after brain biopsy].

A 76-year-old woman with a 7-year history of dementia presented to our hospital with generalized convulsive seizure for the first time. Contrast-enhanced brain magnetic resonance imaging revealed leptomeningeal enhancement mainly in the right occipital lobe and multiple lobar microbleeds in the bilateral cerebral and cerebellar subcortex. No white matter lesions were observed. A brain biopsy of the right parieto-occipital lobe revealed cerebral amyloid angiopathy (CAA). White matter lesions appeared in the right parieto-occipital lobe three days after the biopsy, and we considered inflammatory CAA. Three courses of methylprednisolone pulse followed by oral prednisolone therapy gradually reduced leptomeningeal and white matter lesions. An apolipoprotein E genotype investigation identified the ε2/ε3 genotype. In patients with inflammatory CAA, a risk of exacerbation should be considered after brain biopsy, in which the ε2 allele might play a role.

Possible Cerebral Vasculitis in a Case with Rheumatoid Arthritis.

Cerebral rheumatoid vasculitis (CRV) is a rare, fatal, and diagnostically challenging disorder. We herein report an 81-year-old woman with a 4-year history of rheumatoid arthritis who presented with a fever, progressive disturbance of consciousness, high level of rheumatoid factor, and hypocomplementemia. The enhancement of the perforating branches in the left middle cerebral artery led us to suspect CRV. A brain biopsy could not be performed. After we intensified steroid therapy, the size of the cerebral lesions temporarily decreased. However, recurrence in the left frontal lobe occurred one month later, and the patient subsequently died. Early intensive treatments may be needed for CRV.

[A case of cerebral venous sinus thrombosis mimicking transient ischemic attack].

A 48-year-old woman with a right-sided headache beginning a month prior to admission presented with sudden-onset right hemiparesis. On admission, she had weakness of the right lower extremity, which disappeared 3 hours after onset. Contrast enhanced brain MRI revealed no parenchymal lesion, while indicated thrombi in the superior sagittal sinus and the right side of the transverse sinus, sigmoid sinus, and internal jugular vein, leading to the diagnosis of cerebral venous sinus thrombosis. Brain perfusion single photon emission computed tomography presented slightly decreased blood flow in the bilateral frontal lobes (left-sided dominant) and the right occipitotemporal lobe. Electroencephalogram showed no abnormal finding. After anticoagulant therapy, thrombi in the venous sinuses decreased and brain blood flow improved. We should consider cerebral venous sinus thrombosis in the case of a patient presenting with symptoms of a transient ischemic attack accompanied with headache. Moreover, the etiology of transient neurological deficits remains controversial.

Trousseau syndrome with intrahepatic cholangiocarcinoma that could be removed radically after endovascular treatment: Report of a case.

BACKGROUND: Trousseau syndrome is a poor prognosis. We report a case of Trousseau syndrome treated by radical resection after endovascular treatment. CASE: A 59-year-old woman presented to our department reporting spontaneous dizziness and pain of the upper abdomen. Magnetic resolution imaging (MRI) showed shower embolization of Brain. Contrast-enhanced computer tomography (CT) showed renal infarction and splenic infarction, and a tumor was observed in the retrohepatic area. On day 9, sudden right side joint prejudice, neglect of left half space, and left hemiplegia were observed. MRI revealed obstruction of the right middle cerebral artery (MCA) perfusion zone. On the same day, endovascular treatment was performed and reperfusion was obtained. We decided on a radical surgery policy because there were a primary lesion and a high risk of new embolism, and no metastasis was seen. DISCUSSION: Trousseau syndrome generally has a poor prognosis, but active treatment should be considered as an option when we can expect the recovery of function.

[A case of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-related disease with human leukocyte antigen (HLA) positivity indicative of neuro-Sweet disease].

A 73-year-old man with a 5-day history of continuous hiccup, fever, and rapidly progressing paraplegia was admitted to our hospital. On admission, he exhibited dysarthria, complete paraplegia, and insentience of both lower limbs. Head and spine MRI showed abnormal, asymmetric lesions in the white matter, basal ganglia, and brainstem, and multiple spinal cord lesions. Test for serum anti-AQP4 antibody was negative. Evaluation of human leukocyte antigen (HLA)-B51 was negative; however, HLA-B54 was positive. Although skin lesions were absent, we considered neuro-Sweet disease and high-dose steroid therapy was initiated. The hiccup disappeared gradually, and he regained the ability to walk with a cane 30 days after the onset. Subsequently, the patient tested positive for serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody. It is important to consider MOG antibody-related disease as potential diagnosis in patients exhibiting clinical features of neuro-Sweet disease except for the absence of skin lesions.

[Guillain-Barré syndrome with refractory optic neuropathy].

A 60-year-old woman with a 3-day history of ataxic gait, blurred vision, and upper extremity paresthesia was admitted to our hospital. She presented with severe visual disturbances (finger counting), ophthalmoplegia, neck weakness, and sensory ataxia. Serum anti-GQ1b antibody, anti-GM3 antibody, and anti-GD3 antibody were strongly positive, which might contribute to the pathogenesis. Since we suspected Guillain-Barré syndrome (GBS), intravenous immunoglobulin therapy (IVIg) and high-dose steroid therapy were administered; however, improvements in her visual acuity were minimal. Additional IVIg and high-dose steroid therapy resulted in limited visual acuity improvements. Therapeutic strategies for patients with GBS and refractory optic neuropathy remain controversial.