Hydrocephalus due to marked enlargement of spinal roots in a patient with chronic inflammatory demyelinating polyradiculoneuropathy

Iwasaki, Atsushi; Kokubun, Norito; Funakoshi, Kengo; Hirata, Koichi; Suzuki, Keisuke

doi:10.1111/ene.14492

Cited by 2 publications

(4 citation statements)

References 15 publications

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“…Mental status decline in CIDP patients is usually considered ancillary to the primary disease with no causal link established between the two [ 5 ]. Newer investigations into cognition in CIDP have demonstrated various mild neurofunctional impairments, specifically with information processing speed, flexibility, and verbal processing.…”

Section: Discussionmentioning

confidence: 99%

“…There is an incredible paucity of information regarding CIDP and cognitive decline. The few reports are largely anecdotal and unsupportive of a direct causal link between CIDP and cognitive impairment [ 5 ]. Aside from neurological manifestations, new studies have delved into a potential link between gastrointestinal dysfunction and CIDP [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

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Chronic Inflammatory Demyelinating Polyneuropathy With Reversible Severe Cognitive Impairment and Gastrointestinal Dysfunction

Patrick,

Bielling,

Postma

et al. 2023

Cureus

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We treated a patient with an unusual case of reversible rapidly progressive cognitive impairment, gastrointestinal dysfunction, and generalized neuromyopathy in chronic inflammatory demyelinating polyneuropathy (CIDP) with optic neuropathy. A man in his 50s presented with a four-month history of rapidly progressive cognitive decline in addition to a six-month history of proximal greater than distal painful muscle weakness, wasting in all extremities, almost complete loss of deep tendon reflexes in his lower extremities, and slow progressive vision loss. Additionally, he had a 90-pound weight loss over the past two years with loss of appetite and ongoing chronic diarrhea. The exam showed muscle weakness and wasting with absent deep tendon reflexes. Initial Saint Louis University Mental Status (SLUMS) exam score was 16/30. Visual acuity was 20/25 with full extraocular movements; optical coherence tomography revealed superior arcuate bundle thinning bilaterally. Gastrointestinal workup proved nonrevealing. Serologic studies for vitamin deficiencies, heavy metals, and autoantibodies were negative. Whipple, Giardia lamblia , and Campylobacter jejuni stool testing were negative. Imaging studies were unremarkable. Nerve conduction studies showed demyelinating sensorimotor peripheral neuropathy. Muscle biopsy was indicative of denervation with scattered myopathic changes; no evidence of inflammatory myopathy nor glycogen or mitochondrial abnormalities was seen. Intravenous immunoglobulin treatment was begun. The patient was started at a dose of 0.75g/kg every three weeks. Following good but incomplete clinical improvement after the first treatment, his dose was increased to 1g/kg every three weeks. He improved remarkably after four months of infusions, scoring 30/30 on SLUMS with a full return of muscle strength and reflexes. Diarrhea remitted. Visual acuity and conduction delay remained unchanged. Symptom timing and dramatic response to immunoglobulins suggest a common immunological mechanism. In light of extensive differential investigations, unremarkable imaging and serology, and no other systemic disease processes, this case plausibly represents a potential new CIDP phenotypic variant.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyneuropathy With Reversible Severe Cognitive Impairment and Gastrointestinal Dysfunction

Patrick,

Bielling,

Postma

et al. 2023

Cureus

View full text Add to dashboard Cite

show abstract

“…2,8 Hydrocephalus, meanwhile, is rarely observed in patients with CIDP and to our knowledge has only been reported in 14 prior cases. 9 The mechanism is unknown, although it has been suggested that hypertrophied spinal nerve roots can result in obstructed CSF flow. 9 Protein-related blockage of the arachnoid villi is another possibility and may have played a role here.…”

Section: Discussionmentioning

confidence: 99%

“…9 The mechanism is unknown, although it has been suggested that hypertrophied spinal nerve roots can result in obstructed CSF flow. 9 Protein-related blockage of the arachnoid villi is another possibility and may have played a role here. 10 Elevated CSF protein is of course not unique to CIDP and can be seen in both acquired and hereditary demyelinating polyneuropathies.…”

Section: Discussionmentioning

confidence: 99%