An unusual neuropathy in a diabetic patient: evidence for intravenous immunoglobin-induced effective therapy.

We report the case of a 68-year old type-2 diabetic male patient who was admitted to hospital for progressive weakness in the right lower limb. Although his metabolic control was good, he lost more than 20 kg of weight. Despite intensive physio- and vitaminotherapy, his neurological condition kept on degrading with a severe amyotrophy and pain of the right thigh.

Sequestrum-like appearance of a multiple sclerosis brain lesion on serial magnetic resonance images.

Using serial magnetic resonance imaging, we monitored an unique lesion of the brain in a 15-year-old girl with clinically definite and laboratory-supported remitting-relapsing multiple sclerosis. During initial phases of the disease course, cystic necrosis around the plaque was observed. Later, remyelination of the central core of the lesion was speculated, as similarities in signal intensity between the core and the normal appearing white matter were partially recovered both on the T1- and the T2-weighted images.

Whipple's disease confined to the central nervous system.

We report a 49-year-old woman with a left parietal lesion, shown on CT and MRI as an isolated ring-enhancing mass. The diagnosis of cerebral Whipple's disease was made by brain biopsy; there were no gastrointestinal symptoms nor periodic-acid Schiff-positive inclusions in the jejunal mucosa. This case illustrates atypical Whipple's disease, confined exclusively to the central nervous system.

[Kufs disease with leukoencephalopathy].

We report a case of adult neuronal ceroid lipofuscinosis (Kufs' disease) with leukoencephalopathy on cerebral scan CT and MRI. A 52 year-old woman presented with partial complex epileptic seizure followed by progressive dementia, cerebellar ataxia, pyramidal and akineto-rigid signs and symptoms. After 6 years of evolution, cerebral stereotactic biopsies showed a diffuse gliosis of the white matter, but no clear demyelination.

[Semiology of bismuth encephalopathy. Comparison with seven personal cases (author's transl)].

The authors analyze 99 well-described cases of bismuth encephalopathy and suggest a clinical syndrome according to three stages of the disease (before, during and after the acute period) and three clinical domains (psychiatry, neurology and neuropsychology). A particular attention concerns the presence of sequellar clinical signs (mnesic functions), sometimes observed one year after interruption of bismuth ingestion.