Title: Syringomylia affecting ADLs
Author: Islam SMA, MD, Resident, Phase-B
Department of Physical Medicine & Rehab.
Bangabandhu Sheikh Mujib Medical University
Shahbag, Dhaka-1000, Bangladesh.
Presenting Complaints: Md. Saiful Islam, 15 years old, school boy, 2nd issue of nonconsanguineous parents, coming from Chandpur, Bangladesh presented with the complaints of
weakness and wasting of both hands (R>L) for 1 years and loss of sensation for last 6 months.
The weakness and wasting was gradually increasing and progressive in nature. At first, he lookfall of some objects from his hands and cannot grip tightly. Thereafter, these are gradually
ascends and feeling of loss of sensation. He took some vitamins irregularly. There was no
relevant past history or positive family history associated with the illness. He was not giving any
history of pain, headache, dizziness, bowel/bladder involvement, fasciculation, fever or trauma.
He had difficulties in performing the activity of daily livings.
Physical Examinations: general Physical examination reveals normal vital signs. Neurological
exam of both upper limbs show reduced muscle bulk, increased muscle tone, muscle power grade III at right hand and grade-IV at left hand. Deep reflexes are reduced. There have been
discrete sensory loss over the both upper limbs, neck, back and chest. Lower limbs reveals
otherwise normal, but with bilateral plantar extensor. Musculo-skeletal examination reveals,
Normal Gait, flexed fingers or claw hand deformity with gross small muscle wasting of both
hands, especially over the thenar, hypothenar and dorsal gutter. No tenderness noted and with
normal full passive range of movement of all joints.
Investigations: Lab. Investigation = S.CK-257 U/L, S. Aldolase 3.1 U/L, other routine
investigations are normal. X-ray Chest P/A view and X-ray cervical spine both a/p and lateral
view are normal. MRI of cervical spine- Chiary-I hind brain malformation with a large syringe in
the cervical and visualized dorsal cord.
Possible Diagnoses: 1. Syringomyelia : Positive points are - hand muscles weakness, wasting,
numbness, reflexes diminished. Negative points are - No pain, headache, dizziness, abnormal
movement, bowel/bladder involvement. 2.CIDP :Positive points are - weakness and wasting,
sensory loss, reflexes diminished. Negative points are - No ascending type, no lower limbs
involvement, plantar extensor. 3. MND : (LMN -Type), Positive points are -weakness and
wasting of distal muscle, reflexes -diminished. Negative points are - sensory loss, no
fasciculation. 4. Muscular Dystrophy: Positive points are - weakness and wasting present,
gradual onset. Negative points are - Reflexes - diminished, sensory loss.
Management: The patient is admitted to BSMMU hospital, Bangladesh ; he has given
Rehabilitation protocol and now under neurosurgery care and waiting for surgery to stop further
spinal cord neuronal damage and to improve function. Surgery aimed for decompression, reestablished drainage procedure and making shunts if required.
Discussion: This case presented does not have a typical uniform presentation. The patient only
presents weakness and wasting of both hands with dissociated sensory loss of both upper limbs.
There was no history of local pain or headache, dizziness or un-co-ordinated movements. Brain
stem signs are common is syringomyelia associated with chiary malformations, which remains
absent in the present study case. There has been no lower limbs involvement. Hand muscle
weakness and wasting can be present with various other conditions as well; and we tried to
exclude other possibilities.
Conclusion: There is no known way so far to prevent this condition, other than avoiding injury
to the spinal cord. This slowly progressing condition over time cause severe disability.
Syringomyelia is not a rare case at PMR setting, but it needs a uniform protocol to manage the
long term patient.
Potential implications: Reporting and discussion
Next steps: Recommendation of rehabilitation and other management protocol
Acknowledgements and disclaimers: I, am Dr. Atiqul Islam as submitting author for this abstract
take full responsibility for its integrity and ethical conduct. I certify that each of the co-authors
listed has reviewed this document and approves its submission with their name as listed in the
order listed. I certify that each of the co-authors has agreed that this form lists any pertinent
conflict of interest issues pertaining to their participation in the described program. My authors
and I agree that, should this abstract be accepted for this meeting, the International Rehabilitation
Form has our permission to store, reproduce, distribute, and otherwise use the abstract and any
presentation we may make; including audio, video, and other media; in any form or format. Full
publications of accepted abstracts must acknowledge first presentation at this meeting.