Teaching NeuroImages Neurology Resident and Fellow Section 2015 American Academy of Neurology Vignette A 17 years old male developed bilateral hand tremulousness weakness and decreased muscle bulk over 6 months ID: 780254
Download The PPT/PDF document "A 17 year old male with tremors and wast..." is the property of its rightful owner. Permission is granted to download and print the materials on this web site for personal, non-commercial use only, and to display it on your personal computer provided you do not modify the materials and that you retain all copyright notices contained in the materials. By downloading content from our website, you accept the terms of this agreement.
Slide1
A 17 year old male with tremors and wasting of both hands
Teaching NeuroImagesNeurologyResident and Fellow Section
© 2015 American Academy of Neurology
Slide2Vignette
A 17 years old male developed bilateral hand “tremulousness”, weakness and decreased muscle bulk over 6 months. Bilateral extremity wasting with sparing of brachioradialis muscles
(Figure 1) was evident. With arms outstretched, distal mini-polymyoclonus (Video) occured.Neurophysiology suggests a pure motor neuropathy (chronic denervative changes in C7-T1 roots). MR spine (dynamic flexion sequences)1 (Figure 2) revealed cervical cord atrophy, dural sac anterior shifting and contrast-enhancing epidural compartment. Ong et al.
©
2015
American Academy of Neurology
Slide3Imaging
Ong et al.
AAC
©
2015
American Academy of Neurology
Slide4Imaging
Ong et al.
***ABBC
D
E
A
©
2015
American Academy of Neurology
Slide5Imaging
©
2015 American Academy of Neurology
Ong et al.
Slide6A case of Hirayama disease presenting with polymyoclonus
The constellation of features described suggest Hirayama disease2, postulated to be secondary to chronic segmental cord ischemia from repeated neck flexion over a tight dural sac.
The small amplitude, repetitive arrhythmic movements which were abrupt and shock-like characterizing mini-polymyoclonus, should not be mistaken for tremors.Ong et al.
©
2015
American Academy of Neurology