myotrophic lateral sclerosis with chronic inflammatory demyelinating polyradiculoneuropathy-like neuropathy: a rare clinical case
- VernacularTitle:Хажуугийн хатангирт хатуурал өвчний үед архаг үрэвсэлт миелингүйдэх полирадикулоневропати төст шинжүүд илэрсэн эмнэлзүйн ховор тохиолдол
- Author:
Mendjargal N
1
;
Enkhjargal M
2
;
Uyngaa B
3
;
Egshiglen N
3
;
Tuvshinchimeg T
3
;
Tovuudorj A
1
Author Information
1. Department of Neurology, Mongolian National University of Medical Sciences
2. Department of Neurology, Mongolia Japan Hospital
3. MDI, Mongolian National University of Medical Sciences
- Publication Type:Case Reports
- Keywords:
Paresis, dysphagia, Awaji Criteria
- From:
Mongolian Journal of Health Sciences
2025;87(3):248-253
- CountryMongolia
- Language:Mongolian
-
Abstract:
Background:Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative
disorder characterized by degeneration of upper and
lower motor neurons, leading to muscle weakness, spasticity, dysarthria,
and dysphagia. Chronic inflammatory demyelinating polyradiculoneuropathy
(CIDP) is an autoimmune-mediated neuropathy that primarily
affects nerve fibers specifically myelin sheets. Clinically, CIDP presents
with distal muscle weakness, prominent sensory disturbances, and diminished
or absent deep tendon reflexes. The co-occurrence of ALS
and CIDP is exceptionally rare and poses significant diagnostic and
therapeutic challenges due to overlapping and distinct clinical features.
A Case:A 44-year-old male presented to the Department of Neurology
at the Mongolia-Japan Hospital, Mongolian National University of Medical
Sciences, with progressive muscle weakness in both upper and
lower extremities, along with dysphagia, especially for solids with frequent
choking episodes. The initial symptoms began in May 2023 with
muscle fasciculations, followed by progressive weakness, initially in the
right upper limb and gradually progressing to the left. By August 2023,
the patient developed bilateral arm weakness, dysarthria, and worsening
dysphagia. From August 2024, episodes of head drop were noted.
A progressive weight loss of 11 kg was recorded since January 2024.
Comprehensive neurological evaluation, including antibody profiling,
electromyography (EMG), and nerve conduction studies (NCS), supported
a diagnosis of amyotrophic lateral sclerosis with chronic inflammatory
demyelinating polyradiculoneuropathy-like neuropathy.
Outcome:One month after hospital discharge, the patient demonstrated improvement
in self-care abilities and increased muscle strength
in both proximal and distal upper limb muscles. Notably, there
was marked improvement in overall clinical status.
Conclusion:To our knowledge, this is the first reported case in Mongolia documenting
the simultaneous presentation of ALS and CIDP-like neuropathy.
Globally, such cases are exceedingly rare. Timely and accurate diagnosis,
along with appropriate treatment, contributed to improved clinical
outcomes and a deceleration of disease progression in this patient.
- Full text:2025052423231130212248-253.pdf
