We report on a case of the altered mental status from hyperammonemia due to a urinary tract infection of urease-producing (urea-splitting) bacteria. A 98-year-old Japanese woman, who had no history of liver cirrhosis or portal hypertension, presented with altered mental status. The cause of the altered mental status was attributed to an elevation of ammonia in her blood. The urine culture grew Proteus vulgaris. Complete recovery occurred with the use of an antibiotic for the urinary tract infection. Among patients with urinary tract infections, but without liver cirrhosis or portal hypertension, production by urea-splitting bacteria and the subsequent tubular reabsorption of ammonia, may result in hyperammonemic encephalopathy.

Angiocentric glioma (AG) is an extremely rare tumor that often develops in adolescents. Awake surgery for AG occurring in the eloquent area has not been reported to date. We report a case involving a righthanded 15-year-old boy with AG. He presented with a first-time generalized tonic-clonic seizure and was rushed to the local hospital. CT of the head indicated a left frontal low-density mass with no calcification. He was subsequently referred to our hospital. Comparison with a CT scan obtained two years prior due to mild head trauma indicated that the lesion showed a trend toward enlargement. The lesion was located in the anterior and lateral portions of the primary motor cortex, and MRI showed homogenous hypointensity on T1-weighted and hyperintensity on T2-weighted images. Contrast-enhanced MRI showed a linear contrast effect. The patient underwent awake surgery with successful intraoperative brain mapping and total resection, and brain function was preserved. Pathological analysis revealed AG. He returned to his normal life and has shown no recurrence without additional treatment for 2 years. Thus, awake surgery for complete tumor resection while preserving brain function is effective and safe even in adolescents with AGs.

Lynch syndrome (LS) is an autosomal dominant disorder caused by mutations in mismatch repair (MMR) genes and is also known to be associated with glioblastomas. The efficacy of immunotherapy for LS-associated glioblastomas remains unknown. Herein, we report a rare case of LS-associated glioblastoma, treated with chemotherapy using immune checkpoint inhibitors (ICI). A 41-year-old female patient presented with headaches and sensory disturbances in the right upper limb for 6 weeks. She had been treated for rectal cancer and had a family history of LS. MRI revealed two ring-enhancing lesions in the left precentral gyrus. She underwent subtotal resection, leading to a pathological diagnosis of isocitrate dehydrogenase wild-type glioblastoma. She received daily administration of (temozolomide, 75 mg/m 2 ) and concurrent radiotherapy (60 Gy) postoperatively. However, the tumor recurred 1 year after the initial treatment. A molecular genetic study showed high microsatellite instability (MSI), and she was treated with pembrolizumab therapy. Disease progression occurred despite six cycles of pembrolizumab therapy and radiotherapy at the dose of 40 Gy. She died due to glioblastoma progression 19 months after the initial treatment. The present case demonstrates that some LS-associated glioblastomas may be resistant to ICI despite high MSI, possibly because of intratumor heterogeneity related to MMR deficiency.

We report a case of a 31-year-old woman with glioblastoma multiforme (GBM) in the pineal region with associated leptomeningeal dissemination and lumbar metastasis. The patient presented with severe headache and vomiting. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhanced tumor in the pineal region with obstructive hydrocephalus. After an urgent ventricular-peritoneal shunt, she was treated by subtotal resection and chemotherapy concomitant with radiotherapy. Two months after surgery, MRI showed no changes in the residual tumor but leptomeningeal dissemination surrounding the brainstem. One month later, she exhibited severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop like metastasis in the lumbar region. Finally she died five months after the initial diagnosis. Neurosurgeons should pay attention to GBM in the pineal region, not only as an important differential diagnosis among the pineal tumors, but due to the aggressive features of leptomeningeal dissemination and spinal metastasis.
Adult ; Brain ; Brain Stem ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Glioblastoma* ; Headache ; Humans ; Hydrocephalus ; Leg ; Low Back Pain ; Lumbosacral Region ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neoplasm, Residual ; Pinealoma ; Radiotherapy ; Vomiting

Brainstem gliomas are not common in adults, and the treatment strategies and their outcomes are limited. Immunotherapy is emerging as a promising new modality for the treatment of these gliomas. Here, we report the first case of brainstem glioma treated with a combination of radiotherapy and autologous formalin-fixed tumor vaccine (AFTV). A 32-year-old man presented with left facial numbness and right hemiparesis, and was referred to our department. MRI and open biopsy indicated brainstem glioma, and he was specifically diagnosed with isocitrate dehydrogenase 1-mutant diffuse astrocytoma of WHO grade II. He was treated with stereotactic radiotherapy followed by AFTV three months later. MRI conducted at 42 months after the combination therapy showed a 91% decrease in tumor volume, and the regression was maintained for 5 years. Thus, combination treatment with radiotherapy and immunotherapy may prove to be a promising alternative for the treatment of brainstem glioma.

Methods: A retrospective multicenter study was conducted. The study participants included 345 patients who underwent surgery for spinal metastases from 2010 to 2020 at nine referral spine centers in Japan. Data for each patient were extracted from medical records. To identify the factors predicting survival prognosis after surgery, univariate analyses were performed using a Cox proportional hazards model. Results: The mean age was 65.9 years. Common primary tumors were lung (n=72), prostate (n=61), and breast (n=39), and 67.8% (n=234) presented with osteolytic lesions. The epidural spinal cord compression scale score 2 or 3 was recognized in 79.0% (n=271). Frankel grade A paralysis accounted for 1.4% (n=5), and 73.3% (n=253) were categorized as intermediate or high risk according to the new Katagiri score. The overall survival rates were -71.0% at 6 months, 57.4% at 12, and 43.3% at 24. In the univariate analysis, Frankel grade A (hazard ratio [HR], 3.59; 95% confidence interval [CI], 1.23–10.50; p<0.05), intermediate risk (HR, 3.34; 95% CI, 2.10–5.32; p<0.01), and high risk (HR, 7.77; 95% CI, 4.72–12.8; p<0.01) in the new Katagiri score were significantly associated with poor survival. On the contrary, postoperative chemotherapy (HR, 0.23; 95% CI, 0.15–0.36; p<0.01), radiation therapy (HR, 0.43; 95% CI, 0.26–0.70; p<0.01), and both adjuvant therapy (HR, 0.21; 95% CI, 0.14–0.32; p<0.01) were suggested to improve survival. Conclusions Surgical indications for patients with Frankel grade A or intermediate or high risk in the new Katagiri score should be carefully considered because of poor survival. Chemotherapy or radiation therapy should be considered after surgery for better survival.

A 70-year-old woman with rheumatoid arthritis underwent above-knee amputation due to osteomyelitis after right total knee arthroplasty. After the surgery, the patient started rehabilitation for wearing a prosthetic leg. However, the patient could not wear the prosthesis by herself because of severe upper limb impairment due to bilateral finger joint deformity and muscle weakness associated with the rheumatoid arthritis. Therefore, physical therapists and prosthetists/orthotists collaborated to determine movements that could be performed, even with muscle weakness, using assistive devices such as a Velcro strip handle with the prosthesis and a prosthetic liner stand. Subsequently, repetitive training was performed in an environment similar to the setting of the patient's prosthesis use at home. Consequently, although no change in upper limb function was observed, the patient had increased independence during prosthesis attachment. As she had difficulty wearing and removing her trousers/underwear while wearing the prosthesis, she performed movements using assistive devices and made changes to the order of movements. Six months after the surgery, she could wear the prosthesis and perform self-care correctly by herself and return home. Therefore, to maximize function that enables independence after amputation, helping patients learn how to put on the prosthesis using a team approach is important.