Skip to main content
PAINWeek 2024 Main banner
Icon Legend
This session is not in your agenda.
This session is in your agenda. Click to remove it
Back

P-122 - Unexpected Stripes in the Herd: A Rare Case of Neuropathy and Weakness.

POSTER ABSTRACT
Location:  Exhibit Hall

    Author/Presenter(s)

  • AS

    Andrew Su, M.S.

    Osteopathic Medical Student
    Touro University California - College of Osteopathic Medicine
    Vallejo, California

  • CE

    Charles J. Enfield, M.S.

    Medical Student, 4th year
    Touro University California College of Osteopathic Medicine
    Kensington, California

  • KL

    Kyaw Lin, D.O.

    Interventional Pain Physician
    Touro University California - College of Osteopathic Medicine
    Vallejo, California

Abstract Title: Unexpected Stripes in the Herd: A Rare Case of Neuropathy and Weakness.

Background: Neurosarcoidosis is a diagnostically challenging disease with an abundance of differentials. Granulomatous lesions characteristic of this condition can vary in size and location and may affect a single cranial nerve, multiple cranial nerves, the hypothalamic-pituitary axis, meninges, neural parenchyma, or peripheral nervous system, resulting in a variety of clinical presentations1. Peripheral neuropathy can be present in 15-20% of patients2. Another presenting symptom is pyramidal weakness, which is present in 20-40% of patients who are eventually diagnosed with neurosarcoidosis3.



We present a case study of a patient with a history of neurosarcoidosis who presents for further evaluation of neuropathy and lower extremity weakness.

Purpose/Objectives: A 50-year-old male with a history of neurosarcoidosis presents for further evaluation of neuropathy and weakness. Initial diagnosis was made from auricular, scalp, and knee skin biopsies done in 2020 and 2022 showing “sarcoidal granulomatous dermatitis”. MRI of the 3rd ventricle, hypothalamus, pons, mesencephalon, and temporal lobes done in 2022 was suggestive of infiltrative/granulomatous disease. Neurology and endocrinology determined neurosarcoidosis was the central neurologic process driving panhypopituitarism, secondary male hypogonadism, central hypothyroidism, and secondary adrenal insufficiency. Given worsening rash and hypopituitarism, more aggressive therapy was discussed and follow-up electromyography was ordered which showed “severe subacute axonal sensorimotor peripheral neuropathy”, typical for neurosarcoid-related peripheral neuropathy but showed no evidence of bilateral lower extremity myopathy, consistent with a previous study. Manual muscle testing revealed the patient was able to move against gravity but not against resistance in bilateral ankle dorsiflexion, plantarflexion, and knee extension, also consistent with a previous exam.


Methods: Patient: 50-year-old male with complex medical history including neurosarcoidosis.

Setting: Outpatient pain management clinic.

Consent: Patient provided verbal consent to use of de-identified patient information for medical research.


Results: Peripheral neuropathy and lower extremity weakness can result from a myriad of etiologies, necessitating a thorough differential diagnosis. Common causes include peripheral neuropathies such as diabetic neuropathy, Guillain-Barré syndrome, and chronic inflammatory demyelinating polyneuropathy. Central nervous system disorders like multiple sclerosis and spinal cord pathologies, including compressive myelopathies and transverse myelitis, can also present with similar symptoms. Additionally, metabolic and systemic conditions such as vitamin deficiencies, thyroid dysfunction, and chronic renal failure are known contributors. Management of these alternative causes involves addressing the underlying condition: for instance, strict glucose control in diabetic neuropathy, immunoglobulin or plasmapheresis in Guillain-Barré syndrome, corticosteroids or immunosuppressants in chronic inflammatory demyelinating polyneuropathy, and surgical or pharmacological intervention for spinal cord issues.



In this case study, the patient's history and diagnostic findings suggest neurosarcoidosis as the underlying cause of his lower extremity weakness. The initial diagnosis of neurosarcoidosis was supported by skin biopsies showing sarcoidal granulomatous dermatitis and MRI findings suggestive of granulomatous disease in the brain. Further evaluation by neurology and endocrinology confirmed central neurological involvement, leading to complications such as panhypopituitarism and secondary endocrine insufficiencies. Electromyography results demonstrating severe subacute axonal sensorimotor peripheral neuropathy, without evidence of lower extremity myopathy, align with typical presentations of neurosarcoid-related peripheral neuropathy. Given the constellation of symptoms, the progressive nature of the disease, and the exclusion of other differential diagnoses through comprehensive testing, neurosarcoidosis remains the most plausible cause for the patient’s lower extremity weakness, warranting targeted therapeutic approaches and continued multidisciplinary management.

Conclusions/Implications for future research and/or clinical care: Neuropathic pain with muscle weakness is complex - pathology at any level of the neuromuscular pathway from neurological disorders, myositides, endocrine dysfunctions, electrolyte disturbances, genetic diseases, medications, toxins, infections, or sarcopenia can be the cause4. Neurosarcoidosis often overlaps symptomatically with more prevalent neurological conditions like multiple sclerosis, making it challenging to differentiate5. Electromyography can assist in determining the cause of the patient’s weakness, but a thorough, targeted history and exam are necessary to direct selection of the appropriate nerves and muscles for study6. Ultimately, neurosarcoidosis is diagnosed by exclusion and confirmed by pathologic finding of granulomatous inflammation2. This case underscores the importance of a comprehensive, multidisciplinary approach in diagnosing and managing neurosarcoidosis, particularly in patients with complex presentations. Through vigilant monitoring, targeted therapy, and coordinated care, better outcomes can be achieved for patients with this condition.

References:

  1. Berntsson SG, Elmgren A, Gudjonsson O, Grabowska A, Landtblom AM, Moraes-Fontes MF. A comprehensive diagnostic approach in suspected neurosarcoidosis. Sci Rep. 2023 Apr 21;13(1):6539. doi: 10.1038/s41598-023-33631-z. PMID: 37085608; PMCID: PMC10121682.

  2. Bradshaw MJ, Pawate S, Koth LL, Cho TA, Gelfand JM. Neurosarcoidosis: Pathophysiology, Diagnosis, and Treatment. Neurol Neuroimmunol Neuroinflamm. 2021 Oct 4;8(6):e1084. doi: 10.1212/NXI.0000000000001084. PMID: 34607912; PMCID: PMC8495503.

  3. Barreras P, Stern BJ. Clinical features and diagnosis of neurosarcoidosis - review article. J Neuroimmunol. 2022 Jul 15;368:577871. doi: 10.1016/j.jneuroim.2022.577871. Epub 2022 Apr 18. PMID: 35523055.

  4. Chan F, Riminton DS, Ramanathan S, Reddel SW, Hardy TA. Distinguishing CNS neurosarcoidosis from multiple sclerosis and an approach to "overlap" cases. J Neuroimmunol. 2022 Aug 15;369:577904. doi: 10.1016/j.jneuroim.2022.577904. Epub 2022 Jun 2. PMID: 35689980.

  5. Larson ST, Wilbur J. Muscle Weakness in Adults: Evaluation and Differential Diagnosis. Am Fam Physician. 2020 Jan 15;101(2):95-108. PMID: 31939642.

  6. Lacomis D. Electrodiagnostic approach to the patient with suspected myopathy. Neurol Clin. 2012 May;30(2):641-60. doi: 10.1016/j.ncl.2011.12.007. Epub 2011 Dec 29. PMID: 22361378.