Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population

Background：Progressive bulbar palsy （PBP） is a classic phenotype ofbulbar onset amyotrophic lateral sclerosis （ALS） with more rapid progression and worse prognosis.However,as an often under-understood variant of ALS,isolated bulbar palsy （IBP） appears to progress more slowly and has a relatively benign prognosis.This study aimed to investigate the natural course and clinical features of IBP in Chinese population and to compare them with those of PBP.Methods：The clinical data of patients with bulbar onset ALS were collected from January 2009 to December 2013.Revised ALS Functional Rating Scale （ALSFRS-R）,forced vital capacity （FVC）,and follow-up evaluation were performed,and the differences in basic clinical features,ALSFRS-R,FVC,and primary outcome measures between IBP and PBP were analyzed.The independent t-test,Chi-square test,Mann-Whitney U-test,and Kaplan-Meier analysis were used.Results：Totally 154 patients with bulbar onset ALS were categorized into two groups,33 with IBP and 121 with PBP.In the IBP group,the male to female ratio was 0.7 to 1.0,and the mean onset age was 58.5 years.The mean duration from the onset was 16.0 months,and the mean ALSFRS-R score was 43.4 at patients＇ first visit to our hospital.In 14 IBP patients performing FVC examination,the mean FVC value was 90.5% and there were only two cases with abnormal FVC.In 26 IBP patients completing follow-up,15 （58%） suffered death or tracheotomy and the mean survival time was 40.5 months.Significant differences were noted in sex ratio,onset age,ALSFRS-R score,upper motor neuron limb signs,pure lower motor neuron （LMN） bulbar signs,FVC,and survival time between IBP and PBP.Conclusions：IBP was evidently different from PBP.which was characterized with the predominance of female,pure LMN bulbar signs,an older onset age,a relative preservation of respiratory function,and a better prognosis.

Skull Base Osteomyelitis: A Rare Cause of Multiple Cranial Nerve Palsies—A Case Report from Ghana

Introduction: Skull Base Osteomyelitis (SBO) is an infectious inflammation of the skull bones that is often caused by malignant otitis externa (MOE) and affects the temporal bone. This condition commonly affects immunocompromised individuals and the elderly, particularly those with a history of diabetes mellitus. Diagnosis is challenging because of non-specific symptoms that lead to late detection and complications. This report discusses a case of SBO with multiple bilateral cranial nerve abnormalities and highlights the diagnostic and management challenges in high-risk individuals with subtle clinical signs. Case presentation: This report describes a 63-year-old patient with hypertension and diabetes who underwent surgical debridement of the left ear due to malignant otitis externa 4 months prior to presentation. The patient presented with significant dysarthria, dysphagia, ptosis of the left eye with double vision, and hearing impairment in the left ear. Examination revealed bilateral CN VI palsy, right CN VII palsy, left CN VIII palsy, and a right CN XII deficit. Initial tests were unremarkable, but a high Fungitell assay and a second review of the CT scan and MRI revealed a pathological process in the base of the skull involving bony structures and cranial nerves bilaterally, which helped diagnose SBO. The patient was subsequently discharged with oral voriconazole and continued his usual medications. The patient requested further management abroad, because he did not notice resolution of his symptoms. Surgical treatment was employed abroad to relieve his symptoms, as he recovered slowly. Conclusion: This case report underscores the importance of a multidisciplinary approach to address SBO. Collaboration between specialists in infectious diseases, otolaryngology, radiology, and neurology plays a pivotal role in achieving an accurate diagnosis and developing a tailored treatment plan. Although SBO may be infrequent, this case report highlights the need to maintain heightened clinical suspicion in high-risk individuals.

Tongguan Liqiao acupuncture therapy improves dysphagia after brainstem stroke

Tongguan Liqiao acupuncture therapy has been shown to effectively treat dysphagia after stroke-based pseudobulbar paralysis. We presumed that this therapy would be effective for dysphagia after bulbar paralysis in patients with brainstem infarction. Sixty-four patients with dysphagia following brainstem infarction were recruited and divided into a medulla oblongata infarction group（n = 22）, a midbrain and pons infarction group（n = 16）, and a multiple cerebral infarction group（n = 26） according to their magnetic resonance imaging results. All patients received Tongguan Liqiao acupuncture for 28 days. The main acupoints were Neiguan（PC6）, Renzhong（DU26）, Sanyinjiao（SP6）, Fengchi（GB20）, Wangu（GB12）, and Yifeng（SJ17）. Furthermore, the posterior pharyngeal wall was pricked. Before and after treatment, patient swallowing functions were evaluated with the Kubota Water Test, Fujishima Ichiro Rating Scale, and the Standard Swallowing Assessment. The Barthel Index was also used to evaluate their quality of life. Results showed that after 28 days of treatment, scores on the Kubota Water Test and Standard Swallowing Assessment had decreased, but scores on the Fujishima Ichiro Rating Scale and Barthel Index had increased in each group. The total efficacy rate was 92.2% after treatment, and was most obvious in patients with medulla oblongata infarction（95.9%）. These findings suggest that Tongguan Liqiao acupuncture therapy can repair the connection of upper motor neurons to the medulla oblongata motor nucleus, promote the recovery of brainstem infarction, and improve patient＇s swallowing ability and quality of life.