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[A case of chronic inflammatory demyelinating polyradiculoneuropathy with nocturnal myoclonus]
1Division of Neurology, Tokyo Metropolitan Neurological Hospital.
Abstract:
A 43-year-old man began to notice progressive tingling sensation in his legs and unsteadiness in walking in 1983. His symptoms diminished with prednisolone treatment. In 1986, the same symptoms developed again and abated with prednisolone and plasmapheresis. In 1988 he was admitted because of progression of the symptoms in the legs. Neurological examinations showed mild muscle atrophy and weakness in the legs, loss of the deep reflexes, and impairment of cutaneous and deep sensation of the extremities. Heel-knee test was unsteady, and his gait was wide-based. In addition, Babinski and Chaddock signs were positive bilaterally. He had spontaneous myoclonic jerks of his legs, so-called "tonic triple flexion". These jerks were particularly evident during sleep, and were considered to be "nocturnal myoclonus". The same jerks were provoked by painful stimuli or Babinski's, Marie-Foix's maneuver. These jerks were considered to be so-called "spinal automatism" of spinal or brainstem origin. The latencies of VEP on both sides were prolonged and ABR showed abnormalities indicative of brainstem lesion. There were no abnormalities in cranial and spinal MRI. Our case was proven to have central nervus system lesions in addition to findings of chronic inflammatory demyelinating polyradiculoneuropathy.
Insights
This case study highlights a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who also developed central nervous system lesions. The study details the neurological symptoms and diagnostic findings in this complex presentation.
Area of Science:
- Neurology
- Immunology
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder affecting peripheral nerves.
- Central nervous system (CNS) involvement in CIDP is rare but can complicate diagnosis and management.
Observation:
- A 43-year-old male presented with progressive leg tingling, unsteadiness, and sensory/motor deficits consistent with CIDP.
- Neurological examination revealed muscle atrophy, weakness, areflexia, sensory impairment, and positive Babinski/Chaddock signs.
- The patient experienced myoclonic jerks, described as spinal automatism, and abnormal brainstem auditory evoked potentials (ABR) and visual evoked potentials (VEP).
Findings:
- Despite typical CIDP peripheral nerve findings, the patient exhibited CNS lesions.
- Cranial and spinal magnetic resonance imaging (MRI) showed no abnormalities, underscoring the diagnostic challenge.
- The combination of peripheral neuropathy and central neurological signs suggests a complex autoimmune process.
Implications:
- This case underscores the importance of considering CNS involvement in atypical CIDP presentations.
- Further research is needed to understand the mechanisms and optimal treatment for CIDP with CNS lesions.
- Accurate diagnosis and management are crucial for improving patient outcomes in such complex neurological conditions.