好色先生

好色先生

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Abstract Details

A protean case of neurolymphomatosis
Neuro-oncology
P2 - Poster Session 2 (5:30 PM-6:30 PM)
7-006
Describe a challenging case of a patient with upper and lower motor neuron signs
A 66 year old man presented with chronic progressive pain and weakness of the right arm and leg, followed by diplopia and facial weakness. Exam revealed a right cranial nerve III palsy, left cranial nerve VI palsy, peripheral right cranial nerve VII palsy, atrophy and weakness of the right arm and hand, hyporeflexia throughout the right arm, hyperreflexia throughout the left arm and leg with a Hoffman sign on the left, and diffuse fasciculations sparing the tongue.
Serum and CSF studies were notable only for an elevated GM1 IgM. Brain, cervical and lumbar spine MRIs showed C4-C6 spondylosis and stenosis that alone could not explain his picture. EMG/NCS showed diffuse denervation with neurogenic changes most prominent in the right arm and thoracic paraspinal muscles, with normal conduction studies. Given progressive upper and lower motor neuron signs with electrodiagnostics worrisome for motor neuron disease or an unclear immune-mediated neuropathy, he underwent a course of IVIG without improvement.

Repeat CSF flow cytometry and cytology were concerning for lymphoma. While initial scans and serum studies were negative for malignancy, PET found a right adrenal mass that, on biopsy, unveiled a diffuse large B-cell lymphoma. Repeat MRI of the brain and spine revealed multiple enhancing lesions along multiple nerve roots, the hypothalamus, globus pallidus and corpus callosum. Ultimately, he began systemic and intrathecal chemotherapy with subsequent improvement in exam.

This patient's complex presentation with upper and lower motor neuron signs, and multiple cranial neuropathies, made for a challenging diagnosis. While cervical stenosis, motor neuron disease, and immune-mediated neuropathy were entertained as diagnoses, ultimately a treatable cancer was discovered. This reinforces the importance of avoiding anchoring bias when an evolving clinical picture does not fit prior diagnoses.

Authors/Disclosures
David Valentine, MD (West Coast Neurology)
PRESENTER
No disclosure on file
Andreas N. Neophytides, MD No disclosure on file
Alexander Allen, MD (NYU Center for Men's Health) No disclosure on file
No disclosure on file
Arielle M. Kurzweil, MD, FAAN (NYU) Dr. Kurzweil has nothing to disclose.