[Facial paralysis after temporal bone trauma]. / Paralysies faciales après fracture de l'os temporal.

OBJECTIVES: To evaluate functional outcome in 64 cases of facial paralysis following temporal bone fracture and discuss decisive arguments leading either to medical treatment or surgical management. METHODS: Sixty-four patients suffering from post-traumatic facial paralysis were managed between 1995 and 2003: 38 (59%) were given medical treatment and 26 (41%) underwent surgery. A combined middle fossa and transmastoid approach was mostly used (58%). Electrophysiological testing and CT scan results were the main points of the decision algorithm. RESULTS: Electroneuromyography seems to be the most accurate exploration for guiding treatment. Good results (grades I to II on the House and Brackmann scale) were obtained in 63% of cases after medical management and in 39% of cases after surgical treatment. Grades III or IV were obtained in 13% of medically-treated patients and 42% of surgically-treated patients. CONCLUSION: Management of facial paralysis following temporal bone fracture in accordance with electrophysiological testing (evoked EMG) together with CT scan findings enabled accurate indications for surgical treatment. A good grade I or II result can be expected after medical management. A grade III is at best reached after nerve anastomosis.

Chronic inflammatory demyelinating polyneuropathy associated with dysglobulinemia: a peripheral nerve biopsy study in 18 cases.

The possible occurrence of chronic inflammatory demyelinating polyneuropathy (CIDP) in association with an identified dysglobulinemic status is recognized and a causal relationship between the two has been suggested. We had the opportunity to study 18 patients presenting with CIDP and dysglobulinemia. This was an IgG monoclonal gammopathy (IgG MG) in 8 cases, an IgM monoclonal gammopathy (IgM MG) in 8, an IgG-IgM biclonal gammopathy in 1 case and an IgM monoclonal cryoglobulinemia in another. A peripheral nerve biopsy specimen was available for all patients and the morphological findings in these specimens in the cases of CIDP with IgG MG or cryoglobulin did not differ from those without, whereas characteristic features were observed in the cases of CIDP with IgM MG and anti-myelin associated glycoprotein activity.

Guillain-Barré syndrome occurring in two women after ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus.

We report two women who presented with a Guillain-Barré syndrome just after a ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus. One had characteristic clinical signs and the other had major motor involvement. At neurophysiologic investigations, one had typical demyelinating neuropathy whereas the second had mainly axonal degeneration. At ultrastructural examination of a peripheral nerve biopsy, features of macrophage-associated demyelination were present in both nerve specimens, thus confirming the diagnosis of acute inflammatory demyelinating polyneuropathy, i.e., Guillain-Barré syndrome. Prominent axonal involvement was also present in the motor nerves of the second patient. Insulin therapy had to be permanently continued and these two cases are quite different from the transient diabetes sometimes observed in certain cases of Guillain-Barré syndrome. Both the latter and insulin-dependent diabetes mellitus probably have auto-immune mechanisms. It is likely that in our two patients both auto-immune diseases were triggered by a common event. Such cases of Guillain-Barré syndrome have to be distinguished from other acute diabetic neuropathies.

Acute painful diabetic neuropathy: two patients with recent insulin-dependent diabetes mellitus.

Two young men developed an acute painful peripheral neuropathy a few weeks after being diagnosed to suffer from an insulin-dependent diabetes mellitus. In both cases, peripheral nerve biopsy exhibited a few features of acute axonal degeneration. Additionally, in the first case there was a lymphocytic infiltrate around an endoneurial capillary, and in the second case there were several mast cells in the endoneurium of every fascicle examined. A few months later, the acute pain had disappeared in both cases. Only a few cases of acute painful diabetic neuropathy have been reported so far. A vascular origin seems unlikely and metabolic disorders are probably due to a contemporary severe weight loss. An auto-immune mechanism is an alternative explanation.