Eyelid ptosis and muscle weakness in a child with Kawasaki disease: a case report

Background Kawasaki disease (KD) is an acute febrile vasculitis that often occurs in children under 5 years. Ptosis and muscle weakness associated with KD are rarely documented. Case presentation We present a case of KD with eyelid ptosis and muscle weakness in a 3-year-old boy. At admission, grade IV and grade III muscle strength were recorded for upper and lower limbs, respectively. Diminished patellar tendon reflex was noted. Laboratory evaluation showed hypokalemia with the serum potassium concentration of 2.62 mmol/L. Intravenous immunoglobulin (IVIG) and aspirin were initiated immediately accompanied with methylprednisolone for adjunctive therapy. Potassium supplement was administered at the same time, which resulted in the correction of hypokalemia on the 2nd day of admission but no improvement in ptosis and muscle weakness. Neostigmine testing, lumber puncture, electromyography, and cerebral and full spine MRI were performed, which, however, did not find evidence for neural and muscle diseases. On the 5th day, the fever was resolved. On the 6th day, eyelid ptosis disappeared. And on the 14th day, the muscle strength and muscle tension returned to normal, patellar tendon reflex could be drawn out normally, and the boy regained full ambulatory ability. Conclusions KD might affect the neural and muscular systems, and KD complicated with eyelid ptosis and muscle weakness is responsive to the standard anti-inflammatory treatment plus adjunctive corticosteroid therapy.

PROSPECTIVE STUDY OF BEDSIDE ASSESSMENT OF DIABETIC PERIPHERAL NEUROPATHY

Background: Diabetic peripheral sensorimotor neuropathy is one of the most prevalent neuropathic syndromes affecting around 50 % people with diabetes. Its development is gradual with subtle changes hence ignored. Early diagnosis using simple bedside tools is essential. Methods: The prospective study of evaluation of peripheral neuropathy in diabetes involved examination of 500 diabetic patients for neuropathy. Pinprick, vibration sensation, perception of 5.07 Semmes Weinstein monofilament and Achilles tendon reflex were examined and impairment noted. Results: 39 % patients had impairment of perception of Semmes Weinstein monofilament. Loss of pinprick sensation was found in 65.6 % in patients with diabetes for 5-10 years duration. Abnormal tendon reflex was seen in 64.2 % in patients with diabetes move than 10 years. Loss of Vibration (40.2 %), Wasting (16.6 %), loss of pinprick sensation (44.5%) and loss of abnormal tendon reflex (32.1 %) was found to be more common in type II diabetes. Conclusion: Sensorimotor peripheral neuropathy was more common in long standing diabetes mellitus especially with impaired glycaemic control and type II diabetes mellitus.

Case report of restless anal syndrome as restless legs syndrome variant after COVID-19

Background Coronavirus disease 2019 (COVID-19) has a broad spectrum from respiratory and nasopharyngeal symptoms, cerebrovascular diseases, impaired consciousness, and skeletal muscle injury. Emerging evidence has indicated the neural spread of this novel coronavirus. Restless legs syndrome (RLS) is a common neurological, sensorimotor disorder, but highly under diagnosis disorder. Restless anal syndrome as restless legs syndrome variant associated with COVID-19 has been previously not published. We report a case presenting with restless anal syndrome following COVID-19. Case presentation Although a 77-year-old male with COVID-19 improved to normal respiratory function 21 days after admission and treatment of favipiravir 200 mg per day for 14 days and dexamethasone 6.6 mg per day for 5 days, the insomnia and anxiety symptoms remained. Several weeks after discharge, he gradually began to experience restless, deep anal discomfort, approximately 10 cm from the perineal region. The following features were observed in the anal region; urge to move is essential, with worsening with rest, improvement with exercise, and worsening at evening. Colonoscopy revealed internal haemorrhoids without other rectal lesions. Neurological findings including deep tendon reflex, perineum loss of sensory and spinal cord injury, revealed no abnormalities. Diabetes militias, kidney dysfunction and iron deficiency status were not confirmed. Family history of RLS and periodic limb movements were not observed. Clonazepam at 1.5 mg per day resulted in the alleviation restless anal discomfort. Conclusions We reported a case presenting with restless anal syndrome following affection of COVID-19 as restless legs syndrome variant. This case fulfilled 4 essential features of RLS, urge to move, worsening with rest, improvement with exercise, and worsening at evening. To date, no case of restless anal syndrome associated with COVID-19 has been previously published. This case report may reflect the associative impacts of COVID-19 on the neuropsychiatric state. The long-term outcomes of neuropsychiatric conditions should continue to be monitored.

A Case Report on Late-onset Friedrich's Ataxia and Response on Parkinsonism Treatment

Friedrich's ataxia is a progressive neurodegenerative disease that affects the posterior cord of the spinal tract. We present a case of an 83-year-old male with resting tremor and rigidity that had gradually worsened over the past few years. The patient has been diagnosed with Friedrich's ataxia. Unlike typical Friedrich ataxia, this patient does not have a shortened life expectancy. There is a small percentage of atypical patients demonstrate late-onset of disease, isolated spastic paraparesis without ataxia, and retained or exacerbated deep tendon reflex. Although there is no association between Parkinson's disease and Friedrich's ataxia; in our case, treatment of tremor and rigidity improves the patient's quality of life.

The Pathological Features of Common Hereditary Mitochondrial Dynamics Neuropathy

ObjectivesMitofusin 2 and ganglioside-induced differentiation-associated protein 1 are two main mitochondrial dynamics-related proteins. Dysfunction of these two proteins leads to different subtypes of Charcot–Marie–Tooth disease type 2A (CMT2A) and CMT2K. This study aims to report the pathological difference between CMT2A and CMT2K in a large cohort.MethodsThirty patients with molecularly confirmed CMT2A and nine with CMT2K were identified by next-generation sequencing. Sural nerve biopsies were performed in 29 patients.ResultsThe patients with both diseases showed length-dependent neuropathy with distal weakness, sensory loss, and no deep tendon reflex. Optic neuropathy appeared in 3/30 (10%) patients with CMT2A. Tendon contracture appeared in 4/9 (50.0%) patients with CMT2K. Sural biopsy revealed the loss of both myelinated and unmyelinated nerve fibers. Closely packed, irregularly oriented neurofilaments were observed in axons of unmyelinated nerve fibers in both diseases. Another important finding was the ubiquitous presence of smaller, rounded, and fragmented mitochondria in CMT2A and elongated mitochondria in CMT2K in the myelinated and unmyelinated axons.ConclusionThis study confirmed large diversity in phenotypes between CMT2A and CMT2K. Mitochondrial dynamics-related variations can induce different mitochondrial morphological changes and neurofilament accumulation in axons.

Deep Tendon Reflex: The Tools and Techniques. What Surgical Neurology Residents Should Know

The deep tendon reflex (DTR) is a key component of the neurological examination. However, interpretation of the results is a challenge since there is a lack of knowledge on the important features of reflex responses such as the amount of hammer force, the strength of contraction, duration of the contraction and relaxation. The tools used to elicit the reflexes also play a role in the quality of the reflex contraction. Furthermore, improper execution techniques during the DTR assessment may alter the findings and cloud the true assessment of the nervous system. Therefore, understanding the basic principles and the key features of DTR allows for better interpretation of the reflex responses. This paper discusses the brief history of reflexes, the development of the reflex hammer, and also the key features of a reflex response encompassing the amplitude of force needed to elicit a reflex response, the velocity of contraction, the strength of contraction, and the duration of contraction and relaxation phases. The final section encloses the techniques of eliciting DTR in the upper extremities, trunk, and lower extremities, and the interpretation of these reflexes.

A Smart Tendon Hammer System for Remote Neurological Examination

The deep tendon reflex exam is an important part of neurological assessment of patients consisting of two components, reflex elicitation and reflex grading. While this exam has traditionally been performed in person, with trained clinicians both eliciting and grading the reflex, this work seeks to enable the exam by novices. The COVID-19 pandemic has motivated greater utilization of telemedicine and other remote healthcare delivery tools. A smart tendon hammer capable of streaming acceleration measurements wirelessly allows differentiation of correct and incorrect tapping locations with 91.5% accuracy to provide feedback to users about the appropriateness of stimulation, enabling reflex elicitation by laypeople, while survey results demonstrate that novices are reasonably able to grade reflex responses. Novice reflex grading demonstrates adequate performance with a mean error of 0.2 points on a five point scale. This work shows that by assisting in the reflex elicitation component of the reflex exam via a smart hammer and feedback application, novices should be able to complete the reflex exam remotely, filling a critical gap in neurological care during the COVID-19 pandemic.