Primary amelanotic leptomeningeal melanocytosis mimicking subarachnoid hemorrhage: a case report of the deceptive masquerader - Takeaways - MDSpire

Primary amelanotic leptomeningeal melanocytosis mimicking subarachnoid hemorrhage: a case report of the deceptive masquerader

  • By

  • Yu-Chung Juan

  • John S. Kuo

  • Chun-Chung Chen

  • Ren-Ching Wang

  • Der-Yang Cho

  • Yu-Kai Cheng

  • April 30, 2026

  • 0 min

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  • 1

    Primary diffuse leptomeningeal melanocytosis (PDLM) is a rare CNS neoplasm that poses diagnostic challenges, especially in its amelanotic form.

  • 2

    A 24-year-old male presented with severe headache and neck stiffness, initially misdiagnosed with subarachnoid hemorrhage (SAH) based on CT findings.

  • 3

    MRI revealed diffuse leptomeningeal enhancement without T1 hyperintensity, complicating the diagnosis and mimicking infectious meningitis.

  • 4

    Histopathology confirmed amelanotic PDLM, highlighting the importance of early biopsy and molecular profiling for timely diagnosis and treatment.

  • 5

    The patient experienced rapid neurological deterioration and succumbed to the disease three months post-biopsy, underscoring the aggressive nature of advanced PDLM.

Original Source(s)

Primary amelanotic leptomeningeal melanocytosis mimicking subarachnoid hemorrhage: a case report of the deceptive masquerader

  • by Yu-Chung Juan, John S. Kuo, Chun-Chung Chen, Ren-Ching Wang, Der-Yang Cho, Yu-Kai Cheng

  • April 30, 2026

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