Case ReportIsolated Hypertrophic Neuropathy of the Oculomotor Nerve
Introduction
Hypertrophic neuropathy is a rare disease. In the literature, it has typically been described as a form of hereditary motor and sensory neuropathy (HMSN) or acquired demyelination neuropathy.1 It is characterized by enlarged nerves secondary to the proliferation of Schwann cells around the axons2 and is associated with the peripheral nervous system.3, 4 The most commonly recognized forms of HMSN, although rare, include Charcot-Marie-Tooth disease, Dejerine-Sottas disease, and Refsum disease. Present in infants or adolescents, patients with HMSN disorders experience muscle atrophy, weakness, and sensory abnormalities in the extremities. Most cases are accompanied by deformities such as high arched feet, hammer toes, and scoliosis.5
Hypertrophic neuropathy involving isolated cranial nerves is even rarer. A total of 7 cases have been reported in the literature.6, 7, 8, 9, 10, 11, 12 Among them, trigeminal nerve involvement has been most the common; in 4 of 7 cases, isolated trigeminal nerve involvement was reported6, 8, 9, 11 whereas there has been 1 case report for seventh nerve10 and 2 case reports for eighth nerve involvement.7, 12 To our knowledge, there has been no report of isolated involvement of hypertrophic neuropathy in oculomotor nerves in the literature. Here, we report a case of isolated hypertrophic neuropathy involving an oculomotor nerve.
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History
A 32-year-old white man with a 5-year history of intermittent right retro-orbital headache and mild right ptosis was referred to the Neurosurgery Clinic by his primary care physician. His mother had initially noticed his ptosis in family photographs, but he did not seek medical attention until experiencing double vision with upward gaze. A magnetic resonance imaging (MRI) of the brain demonstrated a suspicious lesion associated with the right third nerve and prompted neurosurgical consultation (
Discussion
Localized hypertrophic neuropathy involving cranial nerves is rare. There are only 7 case reports of localized hypertrophic neuropathy involving cranial nerves in the literature. The trigeminal nerve was mostly commonly involved followed by cranial nerves 7 and 8.6, 7, 8, 9, 10, 11, 12 There has been no report of isolated involvement of hypertrophic neuropathy in oculomotor nerves. In this report, we describe the first case of localized hypertrophic neuropathy involving an isolated oculomotor
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Cited by (2)
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.