Clinical Neurology最新文献

A 74-year-old man sustained a left femoral neck fracture following a fall on day X. Approximately four hours after the injury, he developed impaired consciousness, which persisted even after osteosynthesis was performed on day X+1. Brain MRI on day X+2 revealed multiple hyperintense lesions scattered in the bilateral cerebral hemispheres on diffusion-weighted imaging (DWI), leading to a diagnosis of cerebral fat embolism (CFE). Despite treatment with methylprednisolone and protirelin, his condition did not improve. Nonconvulsive status epilepticus was treated with levetiracetam; however, consciousness remained impaired. Serial brain MRI revealed progressive confluence of white matter lesions. Hyperintense areas on DWI in the cerebral white matter persisted into the subacute and chronic phases. Together with previous reports, this case highlights the potential association between persistence of DWI hyperintensity approximately one month after onset and poor neurological outcomes in patients with CFE.

A 57-year-old man was rushed to our hospital with acute right hemiplegia. Head MRI revealed high signals in the left motor cortex that did not correspond to vascular control, mainly in the cortex, on DWI. However, serum and cerebrospinal fluid were positive for syphilis antibodies, cerebrospinal fluid protein was elevated, and there was no atrial fibrillation. Head MRA and MRV were normal, so the patient was diagnosed with neurosyphilis and antisyphilis therapy was administered. However, the right hemiplegia did not improve much, so an additional steroid was administered, which resulted in a marked improvement in clinical symptoms. It is thought that the steroid contributed to the improvement of symptoms in this case due to vasculitis caused by Treponema pallidum, an excessive immunological response to the destruction of the bacteria, and cytokine release due to inflammation. Additional administration of steroids may be considered in addition to antisyphilis therapy.

A 67-year-old woman undergoing chemoradiotherapy for breast cancer had fever and impaired consciousness. Brain MRI showed abnormal signals in the bilateral frontal lobes. The patient was diagnosed with a brain abscess. The abnormal signal in the right frontal lobe decreased in size with antibiotic treatment, but the abnormal signal in the left frontal lobe worsened. Cytomegalovirus (CMV) was detected in the cerebrospinal fluid. Following administration of ganciclovir, the abnormal signal in the left frontal lobe decreased in size and consciousness improved. Based on the clinical course, we diagnosed a coexisting brain abscess and CMV encephalitis. However, the patient died of respiratory failure caused due to progression of breast cancer and underwent an autopsy. Pathological findings suggested different etiologies for the left and right frontal lobes. They supported the clinical diagnosis of coexisting brain abscess and cytomegalovirus encephalitis. This case indicated that in immunosuppressed patients, coexisting CMV infection and bacterial infection should be considered.

The patient was a woman in her 70s who was admitted to the hospital for a evaluation due to complaints of forgetfulness and frequent falls. She was diagnosed with neuronal intranuclear inclusion disease (NIID) based on the high signal intensity at the corticomedullary junction on diffusion-weighted imaging (DWI) of the brain MRI, the presence of eosinophilic intranuclear inclusions on skin biopsy, GGC repeat expansion in the NOTCH2NLC gene, and her clinical symptoms. Despite the diagnosis of NIID, cerebrospinal fluid (CSF) biomarkers were positive for Alzheimer's disease (AD). Although cerebral blood flow SPECT did not show findings typical of AD, pathological involvement of both NIID and AD was suspected. Anticipating a rapid progression of cognitive decline, the early identification of these two neurodegenerative diseases was considered significant. CSF biomarkers for AD were found to be useful for understanding the complex underlying pathology in patients with cognitive impairment.

We report a 77-year-old male who presented with acute cerebral infarction in the left posterior central gyrus and left superior parietal lobule, and left supramarginal gyrus and its subcortical white matter, resulting in bilateral agraphaesthesia, bilateral astereognosis, and bilateral limb-kinetic apraxia. It is generally known that limb-kinetic apraxia and sensory dysfunction appear on the contralateral side of the lesion, but in this case, limb-kinetic apraxia and some sensory dysfunction appeared on the ipsilateral upper limb as well as the lesion. This suggests that, similar to Liepmann's theory, hemispheric dominance and interhemispheric connectivity are involved.

The objective was to elucidate the relationship between the Hoehn and Yahr scale (HY) and the MDS-UPDRS Part 3 at the OFF state in patients with Parkinson's disease (PD). We conducted a retrospective natural history study using the Parkinson's Progression Markers Initiative database, a large international observational study. The results showed a parallel increase in MDS-UPDRS Part 3 with HY scale. The MDS-UPDRS Part 3 was estimated to increase by 1.90 per year. The median time to HY deterioration were 11, 140, 84, and 47 months for HY 1 to 4, respectively. This study has quantified the motor symptom progression of PD and provided useful information for future clinical trial planning and health economic evaluation of PD treatment.

Case: An 84-year-old woman. She developed a fever (37-38°C) on April X-9, 2021, and took antipyretics and analgesics, but did not improve. She developed temporal pain and eyelid edema on April X-3, which worsened, so she visited our department on April X.

Medical history: She was undergoing treatment for ulcerative colitis. At the initial visit, her body temperature was 38.7°C, her face was edematous (right dominant), and her mouth was restricted. There was tenderness in the temporal region, but there was no engorgement of the temporal artery. Neurologically, there were no abnormalities in the cranial nerves, limb motor, or sensory systems. There were no signs of meningeal irritation.

Examination: Peripheral blood CRP 26.5 ‍mg/dl, WBC 12,900/mm³, RF positive (260 IU/ml), anti-galactose-deficient IgG antibody positive (146 AU/ml), anti-CCP antibody negative, and CK was within the normal range. There were no abnormalities in the temporal artery ultrasound examination. MRI-T₁WI showed swelling of both temporal and masseter muscles, and STIR high intensity signal (right dominant). Symptoms and MRI findings improved promptly after oral administration of 30 ‍mg/day prednisolone. Based on the above, the patient was diagnosed with acute masticatory muscle myositis. Reports of acute masticatory muscle myositis in humans are rare.

A 72-year-old man developed herpes zoster ophthalmicus (HZO), involving the ophthalmic branch of the right trigeminal nerve. The rash completely resolved after a 7-day course of oral antiviral therapy. However, 21 days after the onset of the rash, he developed diplopia. A further 8 days later, he experienced visual impairment in the right eye. He was diagnosed with right abducens nerve palsy and retrobulbar optic neuropathy. Gadolinium-enhanced MRI revealed abnormalities around the optic nerve, extraocular muscles (the inferior, medial, and lateral rectus muscles), and the right orbital apex. Cerebrospinal fluid examination was normal, and VZV-DNA was not detected. Treatment with intravenous acyclovir and methylprednisolone led to improvement in visual function within 5 days. The diplopia also completely resolved within approximately 3 months. The pathophysiology was considered orbital apex syndrome caused by ischemia, inflammation, and edema secondary to vasculitis. This case highlights that HZO can cause diverse cranial nerve complications such as diplopia and visual impairment, even after the resolution of skin lesions.