Clinical Neurology最新文献

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.

A 62-year-old woman was admitted to our hospital with severe pain in all extremities and difficulty in moving. Neurological findings included sensory disturbances with severe pain, weakness, and areflexia in all extremities, with positive Spurling and Lasègue signs. Cerebrospinal fluid analysis revealed protein-cell dissociation. A nerve conduction study revealed the appearance of A-waves and loss of F-waves. Lumbar magnetic resonance imaging revealed gadolinium enhancement in the cauda equina. Therefore, the patient was diagnosed with Guillain-Barré syndrome (GBS) with severe radicular pain. Initially, we started and repeated intravenous immunoglobulin with half-intravenous methylprednisolone pulse therapy (IVMP) and added several types of painkillers, such as acetaminophen, pregabalin, and duloxeline; however, the symptoms, especially severe pain, did not improve significantly. We administered IVMP, and severe radicular pain drastically improved on the first day of IVMP. Here, we describe a case of GBS with primary symptoms of radicular pain, in whom IVMP was effective for radicular pain.

We report three cases of neurosarcoidosis presenting with optic neuritis or myelitis that required differentiation from neuromyelitis optica spectrum disorder (NMOSD) due to positive aquaporin-4 antibody (AQP4-Ab) results. Positive AQP4-Ab findings were identified using a cell-based assay (CBA) in one case and enzyme-linked immunosorbent assay (ELISA) in two cases. The positive results obtained by CBA were considered to represent either latent positivity or false positives, whereas all positive results obtained by ELISA were regarded as false positives. All cases showed elevated soluble interleukin-2 receptor levels in both the serum and cerebrospinal fluid, as well as enlarged mediastinal and hilar lymph nodes. One patient responded poorly to biological monotherapies including ravulizumab. Positive AQP4-Ab results caused by neurosarcoidosis should be considered in cases with features that are atypical for NMOSD.

An 83-year-old woman visited our hospital 44 ‍min after losing the ability to speak. She had total aphasia with Glasgow Coma Scale of E4V1M2 but no conjugate deviation, paralysis, or seizures. MRI diffusion-weighted images showed high-signal intensity lesions in the left temporoparietal lobe and pulvinar and aortogenic cerebral embolism in the right hemisphere; however, no occlusive lesions were observed in the cerebral vessels. Hyperacute cerebral infarction could not be ruled out, and an intravenous recombinant tissue-type plasminogen activator (rt-PA) was administered. The patient's history was obtained the next day, and she was found to have alexia, preceding visual symptoms, and lack of memory at the time of her arrival at the hospital. Based on the T₂ star-weighted images and electroencephalogram, the patient was diagnosed with nonconvulsive status epilepticus originating from an old subcortical hemorrhage. Rt-PA did not cause any severe adverse event. Although speed is required during intravenous rt-PA infusion, stroke mimics can be confused with cerebral infarction even if an MRI is performed; therefore, it is necessary to pay attention to these findings.

The patients were a 59-year-old man and a 53-year-old woman, both of whom had preceding fever, followed by impaired consciousness, urinary retention, and unsteadiness while standing. Neurological examination revealed truncal ataxia. Cerebrospinal fluid testing was positive for anti-glial fibrillary acidic protein (GFAP) α antibodies, leading to a diagnosis of GFAP astrocytopathy. Both patients showed improvement following steroid therapy. Somatosensory evoked potential (SEP) in the tibial nerve stimulation demonstrated prolonged N21-P38 conduction times in both cases, suggesting involvement of the posterior columns of the spinal cord. These findings indicate that posterior column dysfunction may contribute to ataxia in GFAP astrocytopathy, and that SEPs may be a useful diagnostic tool for lesion localization in this condition.