Aug 2026· Radiology Case Reports· Vol 21, pp. 5662 - 5665· 0 citations· 15 references
Medicine
TL;DR
An unusual case of a 37-year-old woman in whom cranial neuropathy preceded peripheral demyelinating neuropathy and characteristic MRI findings of cranial nerve hypertrophy is reported, highlighting the importance of considering CIDP in patients with painful ophthalmoplegia and cranial nerve hypertrophy.
Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disorder affecting the peripheral nerves and nerve roots. It typically presents with symmetrical weakness of both proximal and distal muscles. Although cranial nerve involvement is rare, it can occasionally occur before the typical peripheral neuropathy develops. We report an unusual case of a 37-year-old woman in whom cranial neuropathy preceded peripheral demyelinating neuropathy. She initially presented with painful vision loss caused by compressive optic neuropathy due to trigeminal nerve hypertrophy. She later developed lower limb weakness, and characteristic MRI findings of cranial nerve hypertrophy together with persistent demyelinating changes on nerve conduction studies over a 2-year follow-up confirmed the diagnosis of CIDP. This case highlights the importance of considering CIDP in patients with painful ophthalmoplegia and cranial nerve hypertrophy. Early recognition of these atypical presentations may lead to earlier diagnosis, timely treatment, and better outcomes.
The case of a 47-year-old man with long-standing sicca symptoms who developed progressive, asymmetric motor weakness affecting all four limbs without sensory involvement is reported, describing a rare pure motor axonal neuropathy in a patient with Sjögren syndrome.
Alyaa Kheirbek, Mohamad Yousef Almawaz, Y. Alsaadi et al.· International Medical Case R...· 0 citations
Electrophysiological studies revealed a generalized, asymmetric, multifocal demyelinating neuropathy with conduction blocks at atypical, non-compressive sites, consistent with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM).
Ananya Sukhija, Lyubov P. Smertina· Cureus· 0 citations
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disorder characterized by immune dysregulation, microvascular injury, and progressive fibrosis involving the skin and internal organs. Although Raynaud phenomenon and cutaneous sclerosis are the hallmark manifestations, atypical presentations may significa...
Bhargavan Pv, S. Chandran, A. Us et al.· Cureus· 0 citations
Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), also known as Lewis-Sumner syndrome, is a rare subtype of chronic inflammatory demyelinating polyradiculoneuropathy. We report a patient who initially presented with features suggestive of idiopathic brachial plexopathy and was ultimately diagnose...
Min Kyung Park, Min Seok Kang, Hong Bum Park et al.· Journal of Electrodiagnosis...· 0 citations
Introduction Idiopathic intracranial hypertension (IIH) is defined by elevated intracranial pressure in the absence of an intracranial mass or cerebrospinal fluid abnormality and is commonly associated with papilledema. Although cranial nerve involvement may occur, simultaneous facial and vestibulocochlear nerve dysfun...
Rosángela López-Mena, Carolina Rojas-Cruz, Fernando Ruiz-Magaña et al.· Case Reports in Ophthalmolog...· 0 citations
We report an archival temporal bone case from the Otopathology Archives at Johns Hopkins of a man with progressive distal-predominant weakness, sensory loss, areflexia, and bilateral pes cavus, with clinicopathologic features suggestive of a chronic demyelinating peripheral neuropathy. Bilateral temporal bone histopath...
Yassine Balhi, Syed Ameen Ahmad, Amanda M. Lauer et al.· The Laryngoscope· 0 citations
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