ALS Regional Variants (Brachial Amyotrophic Diplegia and Amyotrophic Leg Diplegia): Still A Diagnostic Challenge in Neurology.

PURPOSE: We illustrate three patients with regional amyotrophic lateral sclerosis (ALS) variants and hope to improve accuracy in diagnosis for this scarce group of diseases. CASE REPORT: Amyotrophic lateral sclerosis (ALS) represents a broad spectrum of acquired and inherited neurodegenerative conditions involving the upper and motor neurons. Typical ALS remains a clinical diagnosis that is not hard to diagnose. Still, when it comes to atypical forms of ALS, the physicians may face some difficulties differentiating between atypical forms of ALS and other neurological diseases, such as multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, and spinal muscular atrophy. Both brachial amyotrophic diplegia (BAD) and leg amyotrophic diplegia (LAD) are considered regional variants of ALS. We are here to report two cases of BAD and one case of LAD. All these 3 cases showed progression of the disease after longitudinal follow- up for approximately two years. However, after two years, their disease progressions were slow and confined to their 'regions' of upper or lower limbs. CONCLUSION: BAD and LAD are unique regional variants of ALS with a significantly better prognosis than typical ALS. The phenotypic characteristics of regional ALS variants must be recognized when physicians are to tailor advice on disease progression, disease outcome, drug therapy, and end-of-life planning for patients with ALS or ALS variants.

Reducing Door-to-Puncture Times for Mechanical Thrombectomy in a Large Tertiary Hospital.

Background and Objectives: Endovascular therapy (EVT) for stroke has emerged as an important therapy for selected stroke patients, and shorter times to clot removal improve functional outcomes. EVT requires the close coordination of multiple departments and poses unique challenges to care coordination in large hospitals. We present the results of our quality improvement project that aimed to improve our door-to-groin puncture (DTP) times for patients who undergo EVT after direct presentation to our emergency department. Methods: We conducted time-motion studies to understand the full process of an EVT activation and conducted Gemba walks in multiple hospitals. We also reviewed the literature and interviewed stakeholders to create interventions that were implemented over 4 Plan-Do-Study-Act (PDSA) cycles. We retrospectively collected data starting from baseline and during every PDSA cycle. During each cycle, we studied the impact of the interventions, adjusted the interventions, and generated further interventions. A variety of interventions were introduced targeting all aspects of the EVT process. This included parallel processing to reduce waiting time, standardization of protocols and training of staff, behavioral prompts in the form of a stroke clock, and push systems to empower staff to facilitate the forward movement of the patient. A novel role-based communication app to facilitate group communications was also used. Results: Eighty-eight patients spanning across 22 months were analyzed. After the final PDSA cycle, the median DTP time was reduced by 36.5% compared with baseline (130 minutes (interquartile range [IQR] 111-140) to 82.5 minutes (IQR 74.8-100)). There were improvements in all phases of the EVT process with the largest time savings occurring in EVT decision to patient arrival at the angiosuite. Interventions that were most impactful are described. Discussion: EVT is a complex process involving multiple processes and local factors. Analysis of the process from all angles and intervening on multiple small aspects can add up to significant improvements in DTP times.

Fine margin between crossed sensory and contralateral sensory loss: A case report.

The well-known sensory deficit in lateral medullary syndrome (LMS) involves crossed ipsilateral trigeminal area and contralateral spinothalamic tract area. A 45 year old man was admitted for sudden onset slurring of speech, unsteadiness and sensory complaints. The diagnosis was right lateral medullary syndrome due to right Vertebral Artery occlusion. In addition to typical lateral medullary syndromes, his sensory deficit involved contralateral half of the face, trunk and limbs. We report this atypical sensory presentation and discuss its neuroanatomical correlation.