ABSTRACT
INTRODUCTION: A typically distal and symmetrical, slowly progressive sensorimotor demyelinating neuropathy is caused by monoclonal IgM against myelin-associated glycoprotein (MAG) and SGPG, SGLPG glycolipids in the context of a benign IgM paraproteinemia. We studied a patient with a neuropathy that fulfilled the diagnostic criteria for CIDP in whom IgM kappa anti-MAG/SGPG/SGLPG were detected. OBSERVATION: The patient was a 57-year-old man who had developed a slowly progressive distal sensorimotor neuropathy, involving the lower then upper limbs, with cranial nerves palsies (oro-pharyngo-laryngo territory). ENMG showed a demyelinating neuropathy with a disproportionate slowing of conduction in distal segments of motor and axonal features in the lower limbs. The first routine laboratory analysis revealed negative or normal findings. Several serum protein electrophoreses were normal. The third cerebrospinal fluid examination demonstrated a moderate and late rise in CSF protein level with no cells. Monoclonal IgM-kappa against MAG/SGPG/SGLPG, was detected; anti-MAG antibody titre in the serum was 20 059 BTU (N<1000). A small IgM-kappa paraprotein was identified by immunofixation. Electron microscopy failed to show nerve fibers with widening of outer lamellae of the myelin. There is no clinical improvement after different treatments, immunoglobulins IV, cortisteroids, plasma exchange, rituximab. CONCLUSION: It is not known whether this neuropathy is an atypical form of PNMAG or an CIDP associated with anti-MAG. When ENMG show a disproportionate slowing of conduction in distal segments of motor nerves, one should screen the serum with immunofixation to identify small monoclonal components. If IgM-MGUS is present, search should be undertaken for anti-MAG/SGPG/SGLPG antibodies. Diagnosis enables optimal treatment using, in severe cases, expensive current strategies with immunoglobulins IV, plasma exchange, and corticosteroids, or, in the event of no response, rituximab before resorting to more toxic drugs like cyclophosphamide.
Subject(s)
Antibodies, Anti-Idiotypic/immunology , Antibodies, Monoclonal/immunology , Globosides/immunology , Immunoglobulin M/immunology , Myelin-Associated Glycoprotein/immunology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/immunology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/physiopathology , Sulfoglycosphingolipids/immunology , Disease Progression , Humans , Male , Middle Aged , Neural Conduction/physiology , Peripheral Nerves/physiopathologyABSTRACT
INTRODUCTION: Spontaneous dissection of cervical and visceral arteries are rare and usually associated with an underlying arterial disease. EXEGESIS: The authors report the unusual case of a 50-year-old woman with high blood pressure who presented spontaneous dissection of cervico-cephalic, renal and hepatic arteries and of the descending aorta. She had been taking ergotamine tartrate for ten years for migraine. She also suffered from Raynaud's syndrome worsened by treatment. CONCLUSION: The respective roles of arterial dysplasia, chronic ergotism, renovascular hypertension and migraine are discussed.
Subject(s)
Aortic Dissection/physiopathology , Ergotism/physiopathology , Fibromuscular Dysplasia/physiopathology , Hepatic Artery , Renal Artery , Aortic Dissection/complications , Aortic Dissection/diagnosis , Angiography , Chronic Disease , Ergotamine/adverse effects , Ergotamine/therapeutic use , Ergotism/complications , Female , Fibromuscular Dysplasia/complications , Humans , Hypertension/complications , Hypertension/physiopathology , Magnetic Resonance Imaging , Middle Aged , Migraine Disorders/drug therapy , Raynaud Disease/complicationsABSTRACT
A 67-year old patient with meningoradiculitis due to Borrelia Burgdorferi presented with unilateral trunk dysesthesias and severe asymmetrical abdominal distension. The causes of abdominal wall paralysis are reviewed.
Subject(s)
Abdominal Muscles , Lyme Disease/complications , Paralysis/etiology , Aged , Humans , Male , Radiculopathy/etiologyABSTRACT
Twenty-seven out of 45 patients who had Wallenberg's syndrome were re-examined as out-patients. Nineteen were complaining of pain on the side opposite to that with loss or temperature and pain sensations or trigeminal deficit. The follow-up showed that pain was both intense and disabling and its treatment was disappointing. The most striking finding was the high frequency of painful sequelae to Wallenberg's syndrome, since they affected almost one out of two patients. The occurrence of these painful symptoms, usually after lesion of the spinothalamic tract, is difficult to explain.
Subject(s)
Lateral Medullary Syndrome/complications , Pain/etiology , Arm/physiopathology , Facial Pain/epidemiology , Facial Pain/etiology , Female , Follow-Up Studies , Humans , Incidence , Leg/physiopathology , Male , Middle Aged , Pain/epidemiology , Time FactorsABSTRACT
Two cases of bilateral thalamic glioma in a 70 year-old man and a 8 year-old boy, documented by MRI and pathological data are reported. Such tumors are rare. Early symptoms may be misleading, with intellectual impairment or psychiatric disorders together with a normal CT scan. MRI and pathological findings support the view that bilateral thalamic gliomas represent a particular clinico-pathological entity among thalamic tumors.
Subject(s)
Brain Neoplasms/diagnosis , Glioma/diagnosis , Thalamic Diseases/diagnosis , Aged , Brain Neoplasms/complications , Child , Glioma/complications , Humans , Magnetic Resonance Imaging , Male , Mental Disorders/etiology , Thalamic Diseases/complicationsABSTRACT
A 59-year old man developed subacute tetraparesis following severe sudden neck pain. MRI showed a subdural cervical hematoma. Prothrombin complex activity was low. An unusual coagulopathy after rodenticides exposure was found. Diphenacoum, an effective antagonist of vitamin K1, was present in the patients plasma. Specific medical management led to a complete recovery. Follow-up MRI seventy days later confirmed the complete disappearance of the hematoma.