Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report

Abstract

Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.

Progressive Encephalomyelitis with Rigidity and Myoclonus: A Syndrome with Diverse Clinical Features and Antibody Responses

“…The MRI scans of our patients were normal, as were those of most reported PERM patients with or without supratentorial involvement [3,7,8]. However, PET examinations of 2 patients revealed abnormal signals in several supratentorial regions, suggesting that PET is a more sensitive tool in diagnosing the pathological CNS alterations in PERM.…”

“…There appear to be no prominent clinical differences between seronegative versus seropositive or paraneoplastic versus non-paraneoplastic PERM cases ([1,7,8,9,10] and our patients). Also, PERM patients with different antibodies present with similar clinical features.…”

“…A significant fraction of PERM and SPS patients displays glutamic acid decarboxylase (GAD) antibodies [4,6,7]. Antibodies to other neuronal synapse proteins such as amphiphysin and glycine receptor (GlyR) have also been identified in PERM patients with or without a tumor [2,3,8,9].…”

Myoclonus in the critically ill: Diagnosis, management, and clinical impact

“…Less frequent critical illnesses that can present with myoclonus are HIV encephalopathy (Canafoglia et al, 2003;Kanjanasut et al, 2010) and prion diseases, such as CreutzfeldtJakob disease (CJD) (Binelli et al, 2010;Chen et al, 2010). Myoclonus generated from spinal cord segments can be associated with traumatic spinal cord injury (Calancie, 2006), spinal cord tumors (Massimi et al, 2009), the Stiff-Person syndrome (Meinck et al, 1995), and with the progressive encephalomyelitis with rigidity and myoclonus (PERM) (Kraemer and Berlit, 2008;Kyskan et al, 2013;Shugaiv et al, 2013;Spitz et al, 2004).…”

Thymoma-Associated Progressive Encephalomyelitis with Rigidity and Myoclonus Successfully Treated with Thymectomy and Intravenous Immunoglobulin

“…PERM is sometimes associated with neoplasms. There has been a report of lung cancer-associated paraneoplastic PERM [4] , and 1 case with thymoma [5] .…”