Disclosures: Aileen L. Giordano, MD: Nothing to disclose

Case Description: The patient presented with fevers in the setting of Hodgkin’s Lymphoma. She was diagnosed with secondary Hemophagocytic Lymphohistiocytosis (HLH). She had profound lower extremity weakness, prompting consultation of the physiatry team. Her initial exam revealed 3/5 strength in the upper extremities as well as hypotonia, absence of volitional movement, and abnormal sensation in the lower extremities. Nerve conduction studies of the left lower extremity revealed absent responses on all tests. Electromyography revealed absence of spontaneous activity or volitional motor unit action potentials in all tested lower extremity muscles; electromyography of the deltoid revealed polyphasicity and early recruitment. She was diagnosed with severe sensorimotor axonal polyneuropathy with concurrent myopathy. Five months after onset of weakness, she transitioned to an inpatient rehabilitation facility (IRF).

Setting: Tertiary care hospital

Patient: 24-year-old woman with Hodgkin’s Lymphoma and Secondary Hemophagocytic Lymphohistiocytosis.

Assessment/Results: She was treated with 1 month of comprehensive inpatient rehabilitation followed by ongoing home health therapies. Her rehabilitation was complicated by neurogenic bladder and orthostasis. At last follow-up, her lower extremity strength exam remained unchanged, although she had modest improvements in hand dexterity. Nevertheless, she made significant functional gains, achieving modified independence with bed mobility and many activities of daily living.

Discussion: HLH is a highly morbid syndrome of immune dysregulation, which has rarely been associated with axonal polyneuropathy. Critical illness polyneuromyopathy (CIPNM) is a more common etiology of weakness; CIPNM has variable outcomes, with most recovery occurring within 6 to 12 months. Without a nerve biopsy, it is unclear if this patient’s impairments are the result of isolated CIPNM or are compounded by HLH-associated polyneuropathy. Her very limited recovery certainly raises concern for a confounding HLH-associated polyneuropathy.

Conclusion: This case reports a severe, heretofore irreversible polyneuropathy in a patient with HLH; despite the severity of her neuropathy, the patient benefitted from comprehensive rehabilitation, making significant functional gains.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/rehabilitation-of-profound-sensorimotor-polyneuropathy-in-the-setting-of-hodgkins-lymphoma-with-secondary-hemophagocytic-lymphohistiocytosis-a-case-report/