P.205 Association Between Vein of Galen Aneurysmal Malformation and Hirayama Disease: A Clue into Pathophysiology?

Background: Hirayama Disease (HD) is a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. Vein of Galen aneurysmal malformations (VGAMs) are rare congenital cerebral vascular malformations, consisting of high-flow arteriovenous shunting between a persistent median prosencephalic vein and arterial feeders. Methods: 14 years old boy known for VGAM presented with left-sided HD. His cervical MRI revealed enlarged epidural with anterior, left-ward displacement of the posterior dura and spinal cord. He underwent surgical treatment by laminotomies, along with tenting of an autologous duroplasty to the overlying laminae. Results: We decided to combine epidural venous plexus coagulation with posterior duraplasty and dural fixation using tenting suture which led to a favorable clinical outcome has not been previously proposed in the literature. We hypothesize that in this context, an abnormal vasculature could also predispose to posterior epidural venous plexus engorgement, anterior dural displacement in cervical flexion, and microvascular changes in the anterior spinal arterial circulation, leading to the progressive anterior horn cell ischemia that lead to the clinical phenotype of HD. Conclusions: The association between HD and VGAM in this patient may provide clues with regard to the pathophysiology of HD.

NeuroAid II (MLC901) in Haemorrhagic Stroke

Stroke is a leading cause of death and disability. NeuroAid (MLC601), which originates from Traditional Chinese Medicine, comprises herbal and animal components, and has been shown to improve the functional status of patients after ischaemic stroke. The use of NeuroAid II (MLC901), which comprises only the herbal components of MLC601, in haemorrhagic stroke has not been previously reported. Our patient is a 63-year-old male with a significant stroke risk factor of hypertension. He developed visual field defect, aphasia, unilateral weakness, and hemisensory loss. CT scan showed a left thalamic haemorrhage. In addition to anti-hypertensive therapy and intensive rehabilitation, he was prescribed MLC901. Over a period of 6 months, he had significant improvements in his motor, sensory, and speech function. There were no adverse events, serial brain CT scans showed resolution of the haemorrhage. MLC901 may have a role in post-stroke recovery after intracranial haemorrhage.

Contralateral Strength Training as Preparation for Direct Strength Training in a Patient With Unilateral Foot-Drop Because of Multiple Sclerosis: A Case Report

Objective Direct strength training (DST) is effective in managing unilateral weakness in people with multiple sclerosis (MS). Its feasibility, however, is considerably reduced if one limb is too compromised to train. In this case, contralateral strength training (CST) of the unaffected side to induce a strength transfer to the untrained homologous muscles can help to establish a strength baseline in the weaker limb, eventually allowing direct training. Limited effects for CST, however, have been reported on patient functioning. We tested the effects on dynamometric, electromyographic, and functional outcomes of a sequential combination of CST and DST of the ankle dorsiflexors in a case of MS-related foot-drop. Methods A 56-year-old man diagnosed with relapsing-remitting MS exhibited severe weakness of the right dorsiflexors impairing functional dorsiflexion. The intervention consisted of a 6-week CST of the unaffected dorsiflexors followed by 2 consecutive 6-week DST cycles targeting the weaker dorsiflexors. Results At baseline, the participant could not dorsiflex his right ankle but could do so after CST. Maximal strength of the affected dorsiflexors increased by 80% following CST, by 31.1% following DST-1, and by a further 44.6% after DST-2. Neuromuscular recruitment was found progressively increased, with the largest changes occurring after DST-1. Improvements in mobility and walking speed were also detected, although plantar flexors’ spasticity on the Modified Ashworth Scale increased from 1+ to 2. Conclusion In this case, the sequential combination of CST and DST proved a feasible approach to manage severe unilateral weakness in a patient who was not able, at least initially, to dorsiflex his weaker ankle. In this perspective, CST may prime a minimum gain in strength necessary to allow subsequent direct training.

Cerebral Venous Sinus Thrombosis as a Presentation of COVID-19

Coronavirus disease 19 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We describe the case of a 59-year-old man who presented with headache, hypertension and a single episode of fever with no other symptoms. He subsequently developed unilateral weakness. Computer tomography identified a cerebral venous sinus thrombosis (CVST). A subsequent test for COVID-19 was positive. This is the first report of CVST as a presenting symptom of COVID-19 infection.

Associations of Video Head Impulse Test and Caloric Testing among Patients with Vestibular Schwannoma

Objective To determine relationships between caloric testing (CT) and video head impulse testing (vHIT) among patients with unilateral vestibular schwannoma (VS). To describe the distribution of CT and vHIT measurements and assess associations with tumor size and self-perceived handicapping effects. Study Design Retrospective review. Setting Tertiary referral hospital. Subjects and Methods Subjects were adults with presumed unilateral VS between 2014 and 2017. Interventions were CT and vHIT. Primary outcomes were vHIT value (abnormal <0.8) and CT value (abnormal >25%). Secondary outcomes were tumor size and Dizziness Handicap Inventory scores. Results Fifty-one individuals had complete data for CT and vHIT. The odds of abnormal gain increases by 2.18 for every 10% increase in unilateral weakness on CT (range, 1.44-3.34; P < .001). A significant negative correlation between CT and gain exists ( rs = −0.64, P < .001). Odds of observing saccades increased by 2.68 for every 10% increase in unilateral weakness (range, 1.48-4.85; P = .001). This association was larger in magnitude for overt than covert saccades (odds ratios, 2.48 and 1.59, respectively). Tumor size was significantly associated with an increase in caloric weakness (β = 0.135, P < .001). With every 10-mm increase of tumor size, odds of abnormal gain on vHIT increased 4.13 (range, 1.46-11.66; P = .007). Mean Dizziness Handicap Inventory score was 19.7 (σ = 22), without association to caloric weakness, gain, or tumor size. Conclusion CT and vHIT both effectively assess vestibular function for patients with VS and correlate to tumor size. These findings are important as vHIT has a lower overall cost, improved patient tolerance, and demonstrated reliability.

Monomelic Amyotrophy (Hirayama Disease) with Proximal Upper Limb Involvement: A Case Report

Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. A 28-year-old man presented with insidious-onset, slowly progressive, unilateral weakness and atrophy of his shoulder girdle and arm muscles on right side. A neurological examination revealed weakness and atrophy in his right deltoid, infraspinatus, supraspinatus and triceps muscles. Electromyography demonstrated an active and chronic neurogenic pattern affecting his right C5 and C6 myotomes; magnetic resonance imaging of his cervical spine showed snake eye appearance within the spinal cord. Upper limb proximal form of monomelic amyotrophy is a rare clinical entity with a wide differential diagnosis. Physicians, especially neurologists, should be familiar with this benign condition to avoid inappropriately labeling patients as having amyotrophic lateral sclerosis and other disorders with less favorable outcomes.Journal of National Institute of Neurosciences Bangladesh, 2018;4(1): 63-66

Reevaluating Order Effects in the Binaural Bithermal Caloric Test

Purpose The purpose of this study was to determine whether a significant order effect exists in the binaural bithermal caloric test. Method Fifteen volunteers (mean age = 24.3 years, range = 18–38 years) with no history of vestibular disorder, hearing loss, concussion, or neurological disease underwent caloric testing on 3 occasions. Irrigations were randomized using 8 possible order combinations. The parameters of interest included unilateral weakness, directional preponderance, total response from the right ear, and total response from the left ear. Results Order effects were analyzed using 2 methods. The first analysis was done looking at the 8 possible orders. We also had an a priori established hypothesis that the first irrigation tested would influence the calculation of unilateral weakness more than the other 3 irrigations. To test this hypothesis, the 8 orders were condensed into 4 order conditions based on the first irrigation. The effect of order was determined using analysis of variance tests. Although the first irrigation tended to be the largest, no significant effects were observed. Conclusions This experiment demonstrated that while there is great inter-individual and intra-individual variability in caloric test results, the order of irrigations had no significant effect in the test. Future studies may explore the effects of nonphysiological factors on test results.

Tongue Signs

The tongue is heavily innervated and plays a central role in articulation, swallowing, and tasting. Unilateral weakness is usually well tolerated. Intermittent weakness of the tongue is highly indicative of myasthenia gravis (MG), while progressive weakness and atrophy is typical for amyotrophic lateral sclerosis (ALS). Tongue weakness without atrophy or fasciculations is a sign of upper motor neuron (UMN) or cerebellar involvement. Lingual dysarthria should be differentiated from other types of dysarthria. Tongue tremor is common and may be confused with fasciculation. Inspection of the tongue while resting in the mouth is more telling than a contracted and protruded tongue for the presence of fsciculations. Direct needle examination of the tongue is painful and is rarely needed.