Severe respiratory muscle paralysis and ventilatory failure is rare in chronic inflammatory demyelinating polyneuropathy (CIDP). We report a 14 year child who presented with respiratory failure, bulbar and multiple cranial nerves involvement along with bilateral phrenic nerve paralysis. He was diagnosed with CIDP after electrophysiological evaluation. He required AMBU ventilation for about 4 months (including domiciliary use), after which he recovered significantly. Along with several unusual features of CIDP, this report highlights good example of steady basic intensive care to save lives and rewarding outcome of prolonged respiratory support, provided by AMBU ventilation which is a rather primitive, but inexpensive device.
A 50-year-old strict vegetarian lady presented with progressive gait instability and paraesthesia of all four limbs lasting 8 months. She had spastic ataxic quadriparesis with pan sensory loss below the C3/4 spinal segment, sensory polyneuropathy, prolonged visual evoked potentials and unrecordable somatosensory evoked potentials. Her serum vitamin B12 concentration was 78 pg/ml. Spinal MRI showed diffuse cervicothoracic cord swelling (fig 1A,B) and posterior column contrast enhancement (fig 2A). The bright posterior column in vitamin B12 deficiency myelopathy is known 1 and has been variously described as an inverted V or inverted rabbit ear sign, 2 with these terms describing the shape of the posterior column on post-contrast T1-weighted MRI spine axial sections. Our case represents a severe B12 deficiency myelopathy with involvement of posterior, anterior and pyramidal tracts in the cervicodorsal spinal cord, as was evident on clinical examination. This resulted in contrast enhancement of the posterior and lateral regions of the spinal cord, resulting in a characteristic ''anchor'' shape on axial sections in cervical spine MRI ( fig 2B). The patient recovered completely after 6 months of parenteral vitamin B12 supplementation. REFERENCES 1. Locatelli ER, Laureno R, Ballard P, et al. MRI in vitamin B12 deficiency myelopathy. Can J Neurol Sci 1999;26:60-3. 2. Naidich MJ, Ho SU. Case 87: subacute combined degeneration. Radiology 2005;237:101-5.Figure 1 (A) Sagittal T1-weighted MRI scan of cervical and upper thoracic spinal cord showing diffuse spinal cord enlargement from the C2 to D1 level. (B) Sagittal T2-weighted MRI scan showing diffuse enlargement of the cervicothoracic spinal cord with hyperintense signals from the C1 to D2 level.Figure 2 (A) Sagittal T1-weighted contrast MRI showing diffuse contrast enhancement of posterior columns in the cervical and upper dorsal spinal cord. (B) Axial contrast T1-weighted MRI cervical spine showing intense enhancement of the posterior column of the spinal cord acquiring a characteristic ''anchor'' shape. Images in medicine186
Flexor spasms are involuntary muscle contractions comprising dorsiflexion at the ankle and flexion at the knee and the hip, occurring as a result of nociceptive spinal release reflex. The presence of flexor spasms generally suggests a lesion in the spinal cord. Foot drop is usually seen with lesions of lumbosacral roots, peripheral nerves or muscles. We hereby present a patient with a rare combination of spastic foot drop and flexor spasms due to a brain tumor. The possible underlying pathophysiological mechanisms resulting in flexor spasms due to a cerebral lesion are briefly discussed.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.