065  Paraneoplastic glycine receptor antibody positive progressive encephalomyeli- tis with rigidity and myoclonus with breast cancer

Abstract

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an autoimmune condition character- ised by various neurological symptoms associated with the dysfunction of the brain stem and spinal cord. PERM has been recognised as a paraneoplastic syndrome, its effects due mainly to the cross reaction of onconeural antibodies against the tumour also affecting neuronal cells.Interestingly, PERM can also present as an isolated syndrome not linked to malignancy. It must be noted however, that tumours may present much later than the syndrome and the aggressive nature of PERM may lead to patient mortality before the tumour can be identified clinically. Paradoxically, the treatment of malignancy can induce remission of PERM as will be illustrated in this case report.A 45-year-old lady initially presented with paraesthesia in both lower limbs followed by leg spasms. There was also difficulty with voiding and constipation. All the initial investigations were negative and the pos- sibility of functional neurological syndrome was made. Few weeks later, patient was readmitted and on this occasion transferred to the neuroscience centre. The patient had features of increased reflexes (including clonus) and also exhibited features of autonomic dysfunction such as heat intolerance, tachy- cardia, hypertension and bowel and urinary dysfunction.Glycine receptor positive progressive encephalomyelitis with rigidity and myoclonus (PERM) was diagnosed and investigations including PET scan confirmed an underlying breast cancer. Initial improvement with immunotherapy was sustained after cancer chemotherapy and surgery.saiju.jacob@uhb.nhs.uk

Anti‐aquaporin‐4 immunoglobulin G/anti‐myelin oligodendrocyte glycoprotein immunoglobulin G double‐positive paraneoplastic neurological syndrome in a patient with triple‐negative breast cancer

“…Breast cancer‐related common paraneoplastic syndrome and their associated antibodies include paraneoplastic cerebellar degeneration (anti‐Yo), paraneoplastic retinopathy (anti‐enolase), opsoclonus‐myoclonus syndrome (anti‐Ri), stiff person syndrome (anti‐glutamic acid decarboxylase, anti‐amphiphysin), paraneoplastic sensory peripheral neuropathy (anti‐Hu, anti‐Yo, anti‐Ri) and paraneoplastic limbic encephalomyelitis (anti‐Hu) 1 . Other rare syndromes, including anti‐SEZ6L2 autoimmune cerebellar syndrome, paraneoplastic progressive encephalomyelitis with rigidity and myoclonus, as well as a case of three concurrent paraneoplastic syndromes (amphiphysin‐related stiff person syndrome, transverse myelitis and hypothalamitis), have been reported in patients with breast cancer 3–5 . AQP4 antibody‐ and MOG antibody‐related paraneoplastic disorders have also been reported individually, but there is a lack of data on cases with dual seropositivity of these antibodies.…”

“…1 Other rare syndromes, including anti-SEZ6L2 autoimmune cerebellar syndrome, paraneoplastic progressive encephalomyelitis with rigidity and myoclonus, as well as a case of three concurrent paraneoplastic syndromes (amphiphysin-related stiff person syndrome, transverse myelitis and hypothalamitis), have been reported in patients with breast cancer. [3][4][5] AQP4 antibody-and MOG antibody-related paraneoplastic disorders have also been reported individually, but there is a lack of data on cases with dual seropositivity of these antibodies.…”