[Paraneoplastic Syndromes in Ophthalmology (French version of the article)]. / Syndromes paranéoplasiques en ophtalmologie.

Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Although these syndromes are rare, the clinical manifestations can herald an unsuspected, underlying malignancy. The associated antibodies and histopathology of each syndrome are presented to help in the understanding of these autoimmune phenomena. Outlined in this review article are diagnostic features useful in differentiating cancer-associated retinopathy, melanoma-associated retinopathy, paraneoplastic polymorphous vitelliform maculopathy, bilateral diffuse uveal melanocytic proliferation and paraneoplastic neurologic syndromes such as optic neuropathy, opsoclonus-myoclonus, Lambert-Eaton myasthenia and paraneoplastic cerebellar degeneration.

Paraneoplastic syndromes in ophthalmology.

Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. They are unrelated to local tumor invasion or metastasis and unexplained by nutritional, metabolic, infectious or iatrogenic causes. The presence of antibodies may aid in the diagnosis of a paraneoplastic syndrome, although this is not an absolute requirement. Chorioretinal involvement is seen in CAR (cancer-associated retinopathy) syndrome, MAR (melanoma-associated retinopathy) syndrome, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation. Paraneoplastic neurological syndromes with ophthalmologic involvement include optic neuropathy, opsoclonus-myoclonus, Eaton-Lambert myasthenic syndrome, paraneoplastic cerebellar degeneration (PCD) and rhombencephalitis.

[Intracerebral cysts revealed by extrapyramidal symptoms]. / Kystes intracérébraux révélés par des symptômes extrapyramidaux.

We report 3 cases of intracerebral cyst revealed by an unusual evolution of extrapyramidal symptoms. Diagnosis was based on MRI and the patients were treated by a cystoperitoneal or a cysto-atrial shunt. The surgical procedure was successful in the 3 cases, resulting in the rapid disappearance of symptoms.

[Neuropathies associated with monoclonal IgM anti-MAG antibodies]. / Neuropathies associées à une IgM monoclonale anti-MAG.

Monoclonal IgM anti-MAG associated peripheral neuropathies are part of demyelinating dysimmune peripheral neuropathies. The hematological disease probably does not influence the outcome of the neuropathy. Neuropathies associated with IgM anti-MAG antibodies are predominantly sensory and distal polyneuropathies associated with ataxia, unsteadiness and tremor. The neurophysiological features include a symmetric sensorimotor demyelinating neuropathy with more slowing of conduction in the distal than in the proximal nerve segments, a length-dependence, and a variable degree of denervation. High titers of IgM anti-MAG antibodies confirm the diagnosis. The natural history is mostly slow with mild to moderate functional impairment. However, some patients have a faster evolution associated with a more severe handicap. Immunotherapies studies have failed to demonstrate significant efficacy of these treatments. Furthermore, severe adverse effects are not uncommon with any of these therapies. Thus, the risk of possible severe adverse effects must be balanced against any possible benefits. More research is needed to improve the management of anti-MAG neuropathies: research on treatment, on prognostic factors, and development of specific assessment scales adapted to the particularities of anti-MAG neuropathies.