Lower motor neuron syndrome due to cauda equina hypertrophy with onion bulbs

O’Ferrall, Erin; Gendron, Daniel; Guiot, Marie‐Christine; Hall, Jeffery A.; Sinnreich, Michael

doi:10.1002/mus.23816

Cited by 15 publications

(19 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A pure motor CIDP with pure motor nerve root involvement was reported in a single patient. 15 However, this patient with cauda equina syndrome had radicular pain consistent with sensory root involvement similar to our patients. The patient also likely had involvement of sensory nerve roots proximal to the dorsal root ganglion, resulting in normal sensory nerve conduction studies and denervation changes in lumbosacral root distribution.…”

Section: Discussionsupporting

confidence: 77%

See 1 more Smart Citation

Chronic immune sensorimotor polyradiculopathy: Report of a case series

et al. 2019

View full text Add to dashboard Cite

Introduction Chronic immune sensorimotor polyradiculopathy (CISMP) is a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant affecting both sensory and motor nerve roots without evidence of peripheral nerve demyelination. Methods We report a case series of 9 patients with CISMP, identified from 2 tertiary centers. Clinical, electrodiagnostic, and neuroradiologic features, and treatment responses, were retrospectively reviewed. Results Patients presented with sensorimotor deficits and hypo‐/areflexia, predominantly involving lower extremities. Three had cranial nerve involvement. Electrodiagnostic findings in all patients localized to roots proximal to dorsal root ganglia, without evidence of peripheral nerve demyelination. Cerebrospinal fluid examination revealed an albuminocytologic association. Eight patients exhibited gadolinium enhancement and thickening of multiple spinal nerve roots and/or cranial nerves. All patients demonstrated good responses to immunotherapies. Discussion CISMP is similar to CIDP in many aspects, but lacks typical electrodiagnostic findings of peripheral nerve demyelination. It is important to recognize this unusual and treatable entity. Muscle Nerve 59:658–664, 2019

show abstract

Section: Discussionsupporting

confidence: 77%

“…Both patients had significant improvement with oral prednisone in combination with azathioprine or MMF. A pure motor CIDP with pure motor nerve root involvement was reported in a single patient . However, this patient with cauda equina syndrome had radicular pain consistent with sensory root involvement similar to our patients.…”

Section: Discussionsupporting

confidence: 62%

Chronic immune sensorimotor polyradiculopathy: Report of a case series

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Histologic findings of three cases from the largest series [1] diagnosed with CISP that underwent lumbar sensory rootlet biopsy showed presence of endoneurial macrophages, lack of degenerating profiles, and features of chronic demyelination and remyelination with numerous onion-bulb formations. ese features support primary demyelination of proximal sensory nerve roots, similar to changes observed in the peripheral nerves of patients with CIDP suggesting that CISP may be an additional phenotype along the CIDP spectrum [8][9][10]. However, unlike CIDP, the distal nerve segments are spared in CISP, as evidenced by normal NCS.…”

Section: Discussionsupporting

confidence: 64%

Early Aggressive Immunotherapy Improves Functional Outcome in Chronic Immune Sensory Polyradiculopathy

Koh

Tung

Tan-Yu

et al. 2020

Case Reports in Neurological Medicine

View full text Add to dashboard Cite

Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with severe sensory ataxia, normal motor examination, unsteady gait, and normal nerve conduction studies (NCS). We describe an elderly man who presented with a two-week history of progressive numbness of both legs and recurrent falls. He had hyporeflexia, normal strength, severe proprioceptive, and vibration sense loss in both lower limbs and was unable to stand or walk because of severe sensory ataxia. e NCS and MR scan of the spine were normal. Tibial somatosensory evoked potentials revealed proximal conduction defect and localized the pathology to the lumbar sensory nerve roots proximal to the dorsal root ganglion. Cerebrospinal fluid showed cytoalbuminergic dissociation suggestive of inflammation. CISP was diagnosed; he was given aggressive immunotherapy consisting sequentially of corticosteroids with mycophenolate mofetil and three cycles of intravenous immunoglobulin after which he regained independent mobility. Unlike previous reports where patients presented months-years after symptom onset and improved after single-line immunotherapy, our patient presented fairly acutely and made dramatic improvement only after aggressive combination therapy. We urge physicians to recognize this uncommon neurologic cause of sensory ataxia where early aggressive treatment is crucial for better functional outcomes.

show abstract

“…Эти методы акту-альны в тех случаях, когда речь идет об атипичных вариантах ХВДП [2,3], а также при дифференциаль-ной диагностике с наследственными формами нейро-патий, когда есть несоответствие клинических прояв-лений выявленному генетическому дефекту [4]. В таких диагностически сложных ситуациях рекомен-дуется более широко использовать нейровизуализаци-онные методы обследования [5].…”

Section: том 7 Volunclassified