Unilateral Abducens Nerve Palsy as an Early Feature of Multiple Mononeuropathy Associated with Anti-GQ1b Antibody

Patients with anti-GQ1b antibody syndrome show various combinations of ophthalmoplegia, ataxia, areflexia, or altered sensorium as clinical features. We describe herein a unique case with unilateral abducens nerve palsy as an early feature of multiple mononeuropathy involving dysfunctions of the inferior dental plexus and the ulnar nerve, which was thought to be associated with anti-GQ1b antibody. A 27-year-old man presented with acute-onset diplopia. He subsequently experienced numbness not only in the right lower teeth and gums but also on the ulnar side of the left hand. Neurological examinations revealed dysfunctions of the right abducens nerve, the right inferior dental plexus, and the left ulnar nerve, suggesting multiple mononeuropathy. Serum anti-GQ1b antibody was positive. This is a rare case report of a patient with unilateral abducens nerve palsy as an early feature of multiple mononeuropathy associated with anti-GQ1b antibody. We suggest that anti-GQ1b antibody syndrome should be taken into consideration as a differential diagnosis of acute multiple mononeuropathy if ophthalmoplegia is present unilaterally

Case report: Recovery from refractory myasthenic crisis to minimal symptom expression after add-on treatment with efgartigimod

Myasthenic crisis, a life-threatening exacerbation of myasthenia gravis, is a significant clinical challenge, particularly when refractory to standard therapies. Here, we described a case of myasthenic crisis in which the patient transitioned from refractory myasthenic crisis to minimal symptom expression after receiving add-on treatment with efgartigimod, a novel neonatal Fc receptor antagonist. A 54 years-old woman who was diagnosed with anti-acetylcholine receptor antibody-positive myasthenia gravis experienced respiratory failure necessitating mechanical ventilation. Despite aggressive treatment with plasmapheresis, intravenous immunoglobulins, and high-dose corticosteroids, her condition continued to deteriorate, culminating in persistent myasthenic crisis. Efgartigimod was administered as salvage therapy. Remarkable improvement in neuromuscular function was observed within days, allowing for successful weaning from mechanical ventilation. Over the subsequent weeks, the patient’s symptoms continued to ameliorate, ultimately reaching a state of minimal symptom expression. Serial assessments of her serum anti-acetylcholine receptor antibody titer showed a consistent decline in parallel with this clinical improvement. This case highlights efgartigimod’s potential as an effective therapeutic option for refractory myasthenic crisis, offering new hope for patients facing this life-threatening condition

Prevalence and Clinical Implication of Microbleeds in Dementia with Lewy Bodies in Comparison with Microbleeds in Alzheimer's Disease

Background: Cerebral microbleeds (MBs) have been well investigated in Alzheimer's disease (AD), but not very extensively in non-AD dementias or in dementia with Lewy bodies (DLB). Aims: To elucidate the clinical significance of MBs in DLB. Methods: We compared the prevalence, locations and risk factors for MBs in 59 DLB and 81 AD patients. We visually counted MBs in each of the cortical and subjacent areas (frontal, temporal, parietal and occipital), the basal ganglia and the thalamus, and the brainstem and the cerebellar hemispheres on 1.5-tesla T2*-weighted gradient-recalled-echo MRI images. White matter lesions were semiquantified in fluid-attenuated inversion recovery images according to the Fazekas rating scale. Results: While the prevalence of MBs was comparable, MBs tended to be more abundant in DLB than in AD in all brain areas with the exception of the occipital lobes. The number of MBs was positively associated with the severity of white matter lesions but not with other vascular risk factors in either AD or DLB. The presence of MBs could be associated with cognitive impairment at onset. MB-positive DLB patients showed less impairment on 123I-metaiodobenzylguanidine myocardial scintigraphy (MIBG scintigraphy) images, supporting the notion of an inverse relationship between vascular lesions and Lewy body pathology. Conclusion: It was suggested that an intricate association between Lewy body pathology, AD-type pathologies and vascular lesions seems to be related to the initial symptoms and results of MIBG scintigraphy in DLB

Agraphia of the left hand with dysfunction of the left superior parietal region without callosal lesions

A 28-year-old right-handed man noticed weakness in his legs, three days after an ephedrine overdose. Initial brain magnetic resonance imaging showed lesions in the parietal regions bilaterally. Computed tomography angiography showed segmental and multifocal vasoconstriction of the cerebral arteries. After treatment, clinical and radiological findings resolved, suggesting the patient had reversible cerebral vasoconstriction syndrome with posterior reversible encephalopathy syndrome. However, he had residual agraphia of the left hand. Language testing revealed no difficulties in oral expression, auditory comprehension, understanding of written language, or writing with the right hand. I-123 iodoamphetamine single-photon emission computed tomography showed residual dysfunction in the left superior parietal lobule. There were no apparent signs of other disconnection syndromes or neuroimaging abnormalities in the corpus callosum. We diagnosed left-hand agraphia due to left parietal dysfunction. Our case suggests that left superior parietal dysfunction without callosal lesions is a possible cause of left-hand agraphia. Neural mechanisms for writing with the right or left hand may be separable at the cortical level. Keywords: Agraphia of the left hand, Disconnection, Left superior parietal region, Reversible cerebral vasoconstriction syndrome, Posterior reversible encephalopathy syndrom

Differential Effects of the Factor Structure of the Wechsler Memory Scale-Revised on the Cortical Thickness and Complexity of Patients Aged Over 75 Years in a Memory Clinic Setting

The Wechsler Memory Scale-Revised (WMS-R) is one of the internationally well-known batteries for memory assessment in a general memory clinic setting. Several factor structures of the WMS-R for patients aged under 74 have been proposed. However, little is known about the factor structure of the WMS-R for patients aged over 75 years and its neurological significance. Thus, we conducted exploratory factor analysis to determine the factor structure of the WMS-R for patients aged over 75 years in a memory clinic setting. Regional cerebral blood flow (rCBF) was calculated from single-photon emission computed tomography data. Cortical thickness and cortical fractal dimension, as the marker of cortical complexity, were calculated from high resolution magnetic resonance imaging data. We found that the four factors appeared to be the most appropriate solution to the model, including recognition memory, paired associate memory, visual-and-working memory, and attention as factors. Patients with mild cognitive impairments showed significantly higher factor scores for paired associate memory, visual-and-working memory, and attention than patients with Alzheimer's disease. Regarding the neuroimaging data, the factor scores for paired associate memory positively correlated with rCBF in the left pericallosal and hippocampal regions. Moreover, the factor score for paired associate memory showed most robust correlations with the cortical thickness in the limbic system, whereas the factor score for attention correlated with the cortical thickness in the bilateral precuneus. Furthermore, each factor score correlated with the cortical fractal dimension in the bilateral frontotemporal regions. Interestingly, the factor scores for the visual-and-working memory and attention selectively correlated with the cortical fractal dimension in the right posterior cingulate cortex and right precuneus cortex, respectively. These findings demonstrate that recognition memory, paired associate memory, visual-and-working memory, and attention can be crucial factors for interpreting the WMS-R results of elderly patients aged over 75 years in a memory clinic setting. Considering these findings, the results of WMS-R in elderly patients aged over 75 years in a memory clinic setting should be cautiously interpreted