clinical quiz Oman Medical Journal [2018], Vol. 33, No.
3: 264-265
Bilateral Upper Limb Weakness
Sivakumar Vadivalagianambi1,2* and Venkatraman Indiran3
1
Gemini Scans Aminjikarai Chennai, Tamil Nadu, India
2
Department of Radiodiagnosis, Meenakshi Medical College Hospital and Research Institute, Tamil
Nadu, India
3
Department of Radiodiagnosis, Sree Balaji Medical College and Hospital, Tamil Nadu, India
A RT I C L E I N F O
Article history: Online:
Received: 3 August 2017 DOI 10.5001/omj.2018.49
Accepted: 29 August 2017
A
21-year-old male presented with a six- rate (80 beats/minute). Routine blood workup
month history of bilateral progressive (hemogram, blood sugar, and renal function tests)
hand and forearm muscle weakness and were normal. Plain cervical spine radiographs were
atrophy. He had no sensory symptoms. within normal limits. Figure 1 shows the magnetic
Power in both hands and forearms was 3/5 and resonance images (MRI) of the cervical spine.
4/5, respectively. Weakness and wasting on both
sides were nearly symmetrical, and his tendon Question
reflexes were normal. There were no fasciculations/ 1. What is the diagnosis?
polyminimyoclonus or autonomic disturbances. a. Amyotrophic lateral sclerosis.
He had no past medical history, allergy history, b. Extradural hemangioma.
or relevant family history. Clinical exam revealed c. Hirayama disease.
normal blood pressure (120/80 mmHg) and pulse d. Devic’s disease.
Figure 1: (a, b) Magnetic resonance imaging (MRI) examination in neutral position showed focal reduction
in spinal cord caliber at C6-C7 level. (c) T2-weighted MRI images in flexion position showed anterior
displacement of the posterior dura by a crescentic extradural intermediate signal intensity, representing a
congested epidural venous plexus.
*Corresponding author: ivraman31@gmail.com
Sivakum ar Vadival agi ana mbi et al. 265
Answer cord (like syringomyelia or tumors).4 However, none
c. Hirayama disease. of these diagnoses demonstrate the characteristic
imaging findings described in Hirayama disease.
In Hirayama disease, chronic ischemic changes
DISCUSSION involving the anterior horns of the cord from C5-T1
The patient’s MRI examination in neutral position level occur due to repeated cord flexion within a tight
revealed a focal reduction in spinal cord caliber dural sac.3 Disproportionate growth of the vertebral
at C6-C7 level [Figure 1a and b]. T2-weighted column in comparison with the spinal cord is also
MRI images in flexion position showed anterior an etiological factor. Raised serum immunoglobulin
displacement of the posterior dura by a crescentic E levels and several allergic disorders have been
extradural intermediate signal intensity, which described in association with Hirayama disease.3
represents a congested venous plexus [Figure Repeated tightening of the spinal dural canal during
1c]. Hirayama disease, a type of benign cervical neck flexion leads to an anterior shift of the posterior
myelopathy associated with neck flexion, affects dura, causing spinal cord compression against the
young males and becomes stationary after a vertebral body, multiple episodes of cord ischemia,
progressive course.1 It is insidious in onset with and chronic trauma. This leads to asymmetric
unilateral upper limb weakness and atrophy. lower cervical cord thinning and myelopathy, as
Classically, there is no lower limb involvement, seen on MRI. Segmental, inferior cervical spinal
sensory disturbance, or tendon reflex abnormalities. cord atrophy on neutral neck position MRI has a
Symptoms concerning the pyramidal, spinothalamic, reported sensitivity of 59% and a specificity of 100%
posterior column tracts, or any polyminimyoclonus for Hirayama disease.5 Flexion neck MRI with thin
or autonomic disturbances in Hirayama disease section heavily T2-weighted sequences exquisitely
are rare. This disease may be wrongly diagnosed demonstrates Hirayama disease. This patient was
as an atypical presentation of amyotrophic lateral treated with a cervical collar, which arrests disease
sclerosis, which is a type of motor neuron disease.2 progression by preventing neck flexion.
Hirayama first described this disease in 1959 and
most cases have been reported from Japan and India.3
Hirayama disease has an initial progressive course r ef er ences
with the spontaneous arrest of disease progression 1. Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH.
Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol
several years after onset differentiating it from 1998 Feb;19(2):365-368.
motor neuron disease, which has a progressive, fatal 2. Hirayama K. Juvenile muscular atrophy of distal
upper extremity (Hirayama disease): focal cervical
course. Amyotrophic lateral sclerosis, caused by ischemic poliomyelopathy. Neuropatholog y 2000
degeneration and death of motor neurons, presents Sep;20(Suppl):S91-S94.
with fasciculation, muscle weakness, stiffness, and 3. Tayade AT, Kale SK, Pandey A, Kalantri S. Hirayama
disease. J Neurosci Rural Pract 2010 Jan;1(1):46-48.
muscle atrophy. All voluntary muscles are gradually 4. Anuradha S, Fanai V. Hirayama Disease: A rare disease with
affected in amyotrophic lateral sclerosis with most unusual features. Case reports in Neurological Medicine.
2016 Dec [cited 2017 Aug 27] Article ID 5839761: [about
dying due to respiratory failure. Other clinical 4p.]. Available from: https://www.hindawi.com/journals/
differential diagnoses include the distal form of spinal crinm/2016/5839761/.
5. Chen CJ, Hsu HL, Tseng YC, Lyu RK, Chen CM, Huang
muscular atrophy, post-polio syndrome, multifocal YC, et al. Hirayama flexion myelopathy: neutral-position
motor neuropathy with conduction block, toxic MR imaging findings–importance of loss of attachment.
Radiology 2004 Apr;231(1):39-44.
neuropathy, and structural lesions of the cervical
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