INTRODUCTION: Although various malignancies have been reported in Paraneoplastic encephalomyelitis, 80% of the cases are associated with bronchial cancer, typically small cell lung cancer.
CASE PRESENTATION: We present a 74-year-old lady admitted with complaints of shaking of the whole body, tremors and rolling of the eyes. She had a history of hypertension and chronic bronchitis. She was a chronic smoker for 40 years and had recently been tapering the cigarettes. . Her psychiatric medications were discontinued in the nursing home 9 days prior to this admission and she was started on Ativan. The clinical impression was withdrawal symptoms versus psychosis. In the hospital she was lethargic and agitated, very anxious and afebrile. The patient continued to have tremulous/dyskinetic movements of the jaw, eyes and upper extremities more than the lower extremities. She responded to verbal commands and responded to her name. A CT scan without contrast of brain showed changes related to atrophy and a chest x-ray was negative. She was also started on Ativan for dystonia and anxiety. A clinical suspicion of meningitis vs encephalitis was entertained and a lumbar puncture was done which showed a normal glucose, normal protein, a negative gram stain and mild lymphocytic pleocytosis. Fungal, TB and viral cultures were all negative. VDRL, Cryptococcal antigen and Lime titers were also negative. She was started on vancomycin, Ciprofloxacin and Acyclovir. A clinical impression was that of a partially treated meningitis or aseptic meningitis or encephalitis. A CT scan of chest with IV contrast showed bilateral emphysema and a 2 x 2 cm low attenuation lesion likely representing adenopathy vs prominent pulmonary artery shadow. Her condition kept on deteriorating and was only responsive to verbal commands and her name. Her prognosis was guarded and she died on Jan. 8, 2006. Autopsy showed small (1 cm) nodule of oat cell (small cell) carcinoma in the right upper lobe of the lung with metastasis to 3 hilar and tracheal lymph nodes. The primary site was smaller than the lymph node metastasis. Her lungs also showed extensive emphysema of both upper lobes of lungs. Her brain microscopically showed encephalomyelitis involving bilateral amygdala, basal ganglia and spinal cord compatible with Paraneoplastic encephalomyelitis syndrome.
DISCUSSIONS: Paraneoplastic Encephalomyelitis (PEM) describes patients with cancer who develop multifocal neurologic deficits and signs of inflammation involving multiple areas of the nervous system, resulting in a mixture of symptoms derived from limbic encephalitis, cerebellar degeneration, brainstem encephalitis, myelitis and autonomic dysfunction. The antineuronal antibodies more frequently encountered in PEM are anti-Hu, anti-CRMP5, anti-Zic, and less frequently anti-amphiphysin. All of these antibodies associate with SCLC and may co-occur in the same patient.75% of patients have MRI abnormalities and PEM rarely improves with treatment. Prompt treatment of the tumor might stabilize the disorderSymptoms can precede the diagnosis of cancer in some cases. Symptoms usually progress over the course of weeks to months causing severe neurologic disability as in this case. In fact neurologic impairment may be more debilitating than the associated cancer as in this case. No effective therapeutic approaches have been established. In this case chest x-rays did not reveal the lung tumor, hence the diagnosis of lung tumor with paraneoplastic syndrome was not made. Retrospectively, all the patient's symptoms can now be explained by this paraneoplastic encephalomyelitis syndrome.
CONCLUSION: PEM often heralds small cell carcinoma of lung. However, to make a definitive diagnosis of PEM, demonstration of presence of antineuronal antibodies, as well as absence of a viral infection is crucial.
DISCLOSURE: Muhammad Rehman, None.