Post-thoracotomy neuralgic amyotrophy in a patient with BAP1 tumor predisposition syndrome: Multimodality longitudinal follow-up

Our plain-English summary of Post-thoracotomy neuralgic amyotrophy in a patient with BAP1 tumor predisposition syndrome: Multimodality longitudinal follow-up, by N. Jazebi, N. Dargah-Zada, K. Alter, et al (Muscle & Nerve, 2021).

The study

• Authors: N. Jazebi, N. Dargah-Zada, K. Alter, R. Shah, C.L. Warga, C.D. Bond, D.S. Schrump, T. Lehky
• Journal: Muscle & Nerve
• Published: 2021;64(3):E7-E9
• Type of article: Case report, Letter
• Read it: PubMed · Publisher · Free full text

In plain English

This letter to the editor was a case report about one patient: a 63-year-old man with an inherited (germline) BAP-1 gene mutation who had malignant mesothelioma, melanoma and renal-cell carcinoma. Asbestos exposure was not discussed. Three days after a right thoracotomy with pleurectomy and decortication, the patient developed shortness of breath, right neck pain, forearm numbness and right-hand weakness. A chest X-ray just after surgery showed normal diaphragms, but the day after surgery the right half of the diaphragm was elevated. Doctors diagnosed lower trunk brachial plexopathy (injury to a nerve network to the arm) with phrenic nerve involvement (the nerve that drives the diaphragm). Steroid treatment slightly improved sensory symptoms but did not improve weakness. Over 14 months, repeated nerve tests and MRI scans showed gradual recovery in the arm nerves, and the hand weakness resolved. The diaphragm recovered very little, and mild breathlessness persisted. The authors described the MRI findings as nonspecific but consistent with inflammation. They stated that the role of the BAP-1 mutation in this nerve inflammation is unknown, and that further studies are needed.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report (letter to the editor) with repeated nerve testing, MRI and ultrasound over 14 months
• Who was studied: One 63-year-old man with a germline BAP-1 mutation and malignant mesothelioma, melanoma and renal-cell carcinoma, seen at the National Institutes of Health, Bethesda, Maryland
• Exposure: No asbestos exposure is described. The report concerns a germline BAP-1 mutation and nerve problems after a right thoracotomy with pleurectomy and decortication.
• Main results:
  • Three days after surgery, the patient had shortness of breath, right neck pain, medial forearm numbness, and weakness of the thumb and second and third fingers
  • A chest X-ray taken immediately after surgery showed normal diaphragms. By the next day, the right hemidiaphragm had become elevated.
  • Intravenous methylprednisolone followed by a prednisone taper gave minimal improvement in sensory symptoms and had no effect on weakness
  • At 14 months the right-hand weakness had resolved, but mild shortness of breath and an elevated right hemidiaphragm persisted
  • Follow-up nerve conduction studies showed a gradual increase in median and ulnar motor response amplitudes. For example, Table 1 lists the median nerve motor amplitude as 2.5 mV at 2 months and 8.6 mV at 14 months. Changes in the phrenic nerve were minor.
  • MRI at 2 and 4 months showed enhancement and swelling of the right lower trunk of the brachial plexus. These findings gradually resolved on MRIs at 7, 11 and 14 months.
  • Ultrasound showed no right diaphragm movement at 2 and 11 months, and only minimal movement at 14 months
• The authors' conclusion: The authors concluded that the role of the germline BAP-1 mutation in this nerve inflammation is unknown. They suggested the mutation may have the potential to cause an inflammatory response, and said further studies are needed on early markers of postsurgical inflammatory neuropathy and on BAP-1's role in inflammation.
• Limitations the authors state:
  • The MRI findings were nonspecific
  • The lack of response to steroids may have been due to delayed treatment, or to the need for a stronger immune-modifying treatment
  • The role of the BAP-1 mutation in the patient's brachial plexitis is unknown

Topics

The National Library of Medicine indexes this study under subjects our site covers: Mesothelioma.

Medical terms in this study, explained in our Lexicon: Thoracotomy, BAP1 Tumor Predisposition Syndrome.

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• Histomorphologic spectrum of BAP1 negative melanocytic neoplasms in a family with BAP1-associated cancer susceptibility syndrome — Z. Marušić, M. Buljan, et al. (2015)
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