[Chemotherapy treatment for anti-Hu paraneoplastic syndrome without active malignancy]. / Traitement par chimiothérapie d'un syndrome neurologique paranéoplasique anti-Hu sans cancer actif associé

INTRODUCTION: Anti-Hu associated paraneoplastic neurological syndromes are rare and characterized by poor prognosis. The research and treatment of a related cancer, a small-cell lung cancer most of the time, remains the best therapeutic strategy. CASE REPORT: We describe the clinical course of a paraneoplastic subacute sensory neuronopathy associated with anti-Hu antibodies in a male smoker treated by an early chemotherapy active against a small-cell lung cancer although no tumor could be found at repeated evaluations. In spite of this treatment, the neurological state deteriorated with the appearance of a cerebellar degeneration, and limbic encephalitis which resulted in a loss of autonomy. A small-cell lung cancer was found and treated 65 months after the onset of the neurological symptoms. The treatment of the underlying malignancy, when it can be found, is still considered as the optimal treatment for paraneoplastic neurological syndromes. Although no tumor could be found, we treated our patient with an empirical chemotherapy active against the most frequent malignancy associated to anti-Hu syndrome in a smoker man, without any improvement. CONCLUSION: Active and repeated research for a cancer related to an anti-Hu neurological syndrome and its treatment are undispensable. For our patient without any identified cancer empirical chemotherapy treatment was unable to stop neurological worsening. When no tumor can be identified by conventional imaging techniques, an early FDG-PET scan should be considered and then repeated if normal.

[Late onset forms of myasthenia gravis. Comparison with early-onset myasthenia gravis]. / Formes tardives de myasthénie. Etude comparative avec la myasthénie du sujet jeune.

INTRODUCTION: The incidence of myasthenia gravis appears to be increasing in elderly but few studies have been devoted to late onset myasthenia gravis. PATIENTS AND METHODS: We retrospectively compared myasthenic patients with an age at onset above or below 35 years which were observed in two departments of Neurology from 1980 to 2002. RESULTS: 81 cases were included, 28 of which were late onset myasthenia gravis. The two populations were similar in terms of sex-ratio, clinical symptoms, course of the disease and therapeutic response. There was a trend for older patients to present more frequently at onset with dysphagia and axial or proximal involvement, and to have extra-ocular symptoms more quickly. Antibodies against acetylcholine receptor and striated muscle were statistically more frequent in elder patients. CONCLUSIONS: A late onset is not a factor of poor prognosis in myasthenia gravis and older patients must be treated in the same way than younger ones.

Tricyclics and malignant syndrome.

A clinical and biological pattern similar to that of neuroleptic malignant syndrome is reported in a depressed patient treated with trimipramine, without any concomitant use of neuroleptics. The antidopaminergic properties of this drug, one of the tricyclic antidepressants, may account for this uncommon side effect.

Sleep polygraphic studies using cystomanometry in twenty patients with enuresis.

Polygraphic exploration during sleep using cystomanometry was performed in 20 patients aged 7-17 years with primary (17) or secondary (3) enuresis. In this group of patients, 9 presented with isolated nocturnal enuresis while 11 patients had associated diurnal micturition troubles. During this study we documented 24 episodes of enuresis. There was no disturbance in sleep architecture or correlation between the uncontrolled micturition and any particular state or stage of sleep. Most episodes of enuresis occurred in a unique pattern in which a sudden or progressive intravesical increased pressure was associated with an awakening reaction. From a physiopathologic point of view, our findings are in favor of immaturity of the central system of inhibition of micturition reflex during sleep.

Surgical alternatives to uvulopalatopharyngoplasty in sleep apnea syndrome.

Uvulopalatopharyngoplasty (UPPP) is the surgery most often performed for sleep apnea syndrome (SAS). However, good results with UPPP, demonstrated by polysomnography, have been reported in only 50% of cases. Failure of UPPP may be caused by: 1) bad management of the SAS, which is better treated in some patients with nasal CPAP than with surgery; and 2) an airway obstruction located not only at the palatopharynx (PP) level. Other surgical procedures to enlarge other sites of obstruction are described. Retro-tongue-base-pharynx (RTBP) surgery is emphasized, including mandibular advancement, hyoid bone suspension, and tongue base reduction. Maxillomandibular advancement is the most efficient technique but also the most complicated.

Uptake of retinyl ester in HL-60 cells via the low-density-lipoprotein-receptor pathway.

Newly absorbed retinol is transported in association with chylomicrons and their remnants. In addition, after intake of high doses of retinol, significant amounts are also found in low-density lipoprotein (LDL). As both chylomicron remnants and LDL may be taken up by cells via the LDL receptor, and retinoids inhibit proliferation of some leukaemic cells, we have studied the uptake of retinol in leukaemic cells via the LDL-receptor pathway. HL-60 cells contain saturable binding sites for LDL. The binding of LDL to its receptor has a dissociation constant of about 3.2 x 10(-9) M, and the number of receptors per cell was calculated to be about 2700. Uptake of 125I-LDL by HL-60 cells was increased 2-fold by preincubating the cells with mevinolin. The presence of specific receptors for LDL on HL-60 cells was further confirmed by the finding that exogenous LDL cholesterol was able to up-regulate the ACAT (acyl-CoA: cholesterol acyltransferase) activity of HL-60 cells. We then tested the uptake of retinyl ester in leukaemic cells via the LDL-receptor pathway. HL-60 cells were incubated with LDL or chylomicron remnants labelled with [3H]retinyl palmitate. Uptake of retinyl ester associated with both LDL and chylomicron remnants was observed. Furthermore, the presence of excess LDL decreased the uptake by 75-100%, supporting the hypothesis that the uptake of retinyl ester occurred via the LDL receptor in HL-60 cells.