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Progressive ataxic gait disorder.

Jayantha Rupasinghe1, Ernest Butler

  • 1Department of Medicine, Frankston Hospital, Peninsula Health, Frankston, Victoria, Australia. jayantharup@hotmail.com

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
|December 13, 2006
PubMed
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Paraneoplastic cerebellar degeneration (PCD) is a rare neurological disorder. Early cancer detection is crucial, as neurological symptoms often appear before the tumor is identified, posing a diagnostic challenge.

Area of Science:

  • Neurology
  • Oncology
  • Pathology

Background:

  • Paraneoplastic cerebellar degeneration (PCD) is a neurological syndrome linked to an underlying malignancy.
  • Anti-Yo associated PCD frequently presents with neurological symptoms preceding cancer diagnosis, particularly ovarian and breast cancers.
  • Early diagnosis and treatment of the causative tumor are critical for managing PCD.

Observation:

  • A patient presented with PCD, and a positron emission tomography scan revealed a pathological lymph node in the right axilla, suggesting a secondary tumor of unknown primary.
  • Histopathological examination of the resected lymph node showed a poorly differentiated adenocarcinoma, strongly positive for C-ERB 2 oncoprotein.
  • Despite extensive imaging, including magnetic resonance mammography, the primary breast carcinoma remained undetected.

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Findings:

  • The patient's neurological symptoms did not improve with standard treatments like intravenous gamma-globulin, steroids, and rehabilitation.
  • Diffuse T2 signal hyperintensity in the cerebellum was observed on MRI, potentially serving as a diagnostic indicator for PCD.
  • This case highlights the diagnostic challenges in identifying the primary tumor in PCD, even with advanced imaging techniques.

Implications:

  • The findings underscore the importance of thorough investigation for occult malignancies in patients with paraneoplastic syndromes.
  • C-ERB 2 positivity in metastatic adenocarcinoma may offer clues for primary tumor origin, although a primary breast cancer was not localized in this case.
  • Cerebellar T2 signal changes could be a valuable radiological marker aiding in the diagnosis of PCD.