Trigeminal nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

An unusual clinical manifestation of nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is described. A patient with a 13-year history of CIDP developed diplopia and proptosis. Imaging of the neuraxis showed marked bilateral trigeminal nerve hypertrophy and lumbosacral nerve root hypertrophy. Biopsy of the right infraorbital nerve revealed inflammatory infiltrates and extensive onion bulb formation, consistent with CIDP.

MNGIE neuropathy: Five cases mimicking chronic inflammatory demyelinating polyneuropathy

“…Specifically, four (patients 1-4) had cachexia and gastrointestinal problems, all had leukoencephalopathy, and four (patients 1, 3, 4, and 5) had ophthalmoplegia. Although CNS demyelination 3,9 and extraocular abnormalities 4 have been described in CIDP cases, they are exceedingly rare. In addition, elevated serum lactate is common in MNGIE, whereas it is rare in CIDP.…”

Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…It is not clear, in the latter case, to what extent this results from a Fisher syndrome-like relapse of CIDP, although in a case where anti-GQ1b antibodies were measured these turned out to be negative. 8,37,50 Some of the cases may also be contributed to by mechanical restriction secondary to proptosis cause by hypertrophic nerve enlargement (Table 2), 6,8,22,23 although the proptosis in our third case was not accompanied by any degree of ophthalmoparesis.…”

“…6,8,[22][23][24][25][26][27][28][29][30][31][32][33][34] The neuro-ophthalmological presentation usually occurs in the context of a long history of CIDP (Tables 1 and 2). It may represent peripheral or central effects of the disease and also may precede the onset of limb involvement.…”

“…22 Other treatment options for refractory papilloedema include acetazolamide, optic nerve sheath fenestration, and CSF diversion surgery. 27,46,49 Declaration of interest: authors report no conflicts of interest.…”

Schwann cell-onion bulb tumor of the trigeminal nerve: hyperplasia, dysplasia or neoplasia?

“…Later reports revealed them to be a nonspecific finding related to repeated bouts of demyelination-remyelination that may develop in a number of diseases, including Refsum's disease [10], Krabbe's (globoid cell) leukodystrophy [23], chronic inflammatory polyneuropathy [11], and rarely multiple sclerosis [21,22], diabetic neuropathy [1] and neurofibromatosis type 1 [17,19,24]. Onion bulbs may be produced experimentally by repeated tourniquet application [8], disruption of the perineurial barrier [15], lead exposure or other chemical injury [16,25].…”