CT and MR findings in extramedullary haematopoiesis with biliary system encasement: a case report

Extramedullary haematopoiesis is the production of blood elements outside the bone marrow cavity. In our case computed tomography and magnetic resonance imaging revealed the presence of a rare localization of extramedullary haematopoiesis with encasement of the biliary system in a 59 years-old male Caucasian patient, with chronic myelofibrosis and hepatic failure’s symptomatology. Computed tomography detected the presence of homogeneous hypodense tissue around intra-hepatic bile ducts with minimal contrast enhancement, strongly suggestive for extramedullary haematopoiesis. Magnetic resonance confirmed the presence of a solid tissue surrounding the biliary tree, showing late enhancement after gadolinium administration suggestive for non-active lesion of extramedullary haematopoiesis. Final diagnosis was established by percutaneous biopsy

Progressive Encephalomyelitis with Rigidity and Myoclonus Associated With Anti-GlyR Antibodies and Hodgkin’s Lymphoma: A Case Report

IntroductionA 60-year-old man presented with a 6-month history of low back pain and progressive rigidity of the trunk and lower limbs, followed by pruritus, dysphonia, hyperhydrosis, and urinary retention. Brain and spinal imaging were normal. EMG showed involuntary motor unit hyperactivity. Onconeural, antiglutamic acid decarboxylase (anti-GAD), voltage-gated potassium channel, and dipeptidyl peptidase-like protein 6 (DPPX) autoantibodies were negative. CSF was negative. Symptoms were partially responsive to baclofen, gabapentin, and clonazepam, but he eventually developed severe dysphagia. Antiglycine receptor (anti-GlyR) antibodies turned out positive on both serum and CSF. A plasmapheresis cycle was completed with good clinical response. A PET scan highlighted an isolated metabolically active axillary lymphnode that turned out to be a classic type Hodgkin lymphoma (HL), in the absence of bone marrow infiltration nor B symptoms. Polychemotherapy with ABVD protocol was completed with good clinical response and at 1-year follow-up the neurological examination is normal.BackgroundProgressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and severe neurological syndrome characterized by muscular rigidity and spasms as well as brain stem and autonomic dysfunction. It can be associated with anti-GAD, GlyR, and DPPX antibodies. All of these autoantibodies may be variably associated with malignant tumors and their response to immunotherapy, as well as to tumor removal, is not easily predictable.ConclusionProgressive encephalomyelitis with rigidity and myoclonus has already been described in association with HL, but this is the first case report of a HL manifesting as anti-GlyR antibodies related PERM. Our report highlights the importance of malignancy screening in autoimmune syndromes of suspected paraneoplastic origin