Association of patella alta with worsening of patellofemoral osteoarthritis-related structural damage: data from the Osteoarthritis Initiative.

OBJECTIVES: To determine the association between Insall-Salvati ratio (ISR), a measure of patella alta, and worsening of Magnetic Resonance Imaging (MRI)-based osteoarthritis (OA)-related patellofemoral joint structural damages over 24-month in participants of the Osteoarthritis Initiative (OAI). DESIGN: Using weighted random sampling method, we selected a sample of 500 knees (from 1,677 knees with available baseline and 24-months MRI OA Knee Score (MOAKS) measurements), which is OAI-representative regarding knee OA-related factors (i.e., baseline age, sex, body mass index (BMI), and radiographic Kellgren-Lawrence grading). The ISR was measured in all enrolled knees using baseline sagittal 3T-MRI plane by three radiologists. Baseline and 24-month MOAKS variables for patellofemoral bone marrow lesions (BMLs), cartilage damages, and osteophytes were extracted, and the associations between ISR and 24-month worsening of these 3T-MRI features were evaluated using multivariable regression models. After computing receiver operating characteristic curves, the optimal cutoff point of ISR for indicating worsening of patellofemoral OA was determined. P-values were adjusted for multiple comparisons and false discovery rate (FDR) adjusted P-values were reported. RESULTS: In this longitudinal analysis, 24-month worsening of BML (odds ratio [OR] (95% confidence interval [95% CI]):11.18 (3.35-39.6), adjusted-p-value:<0.001) and cartilage scores (OR:7.39 (1.62-34.71), adjusted-p-value:0.042) in lateral patella was associated with higher baseline ISR. However, higher ISR was not statistically associated with medial patellar or medial and lateral trochlear BML or cartilage scores worsening. We determined the optimal cutoff point of ISR≥1.14 (95% CI: 1.083-1.284) for predicting lateral patellofemoral OA-related structural damages worsening over 24-months (sensitivity:73.73%; specificity: 66.67%). CONCLUSIONS: Given the uncertainly surrounding the results, our overall findings suggest that ISR could be considered as a predictor of lateral patellofemoral OA-related structural damages worsening with the optimal cutoff point of ≥1.14 using knee sagittal MRI measurements.

Rapid efficacy of highly active antiretroviral therapy in a case of HIV myelitis.

An HIV-seropositive patient with severe immunodepression was diagnosed as having HIV myelitis. Plasma and cerebrospinal fluid (CSF) HIV-RNA PCR were, respectively, 4.11 and 5.19log(10). After 1 month of treatment with highly active antiretroviral therapy (HAART), there was clinical recovery and both plasma and CSF HIV viral load had decreased considerably. This dramatic improvement was associated with a high concentration of antiviral drugs in the CSF, suggestive of the direct efficacy of HAART on HIV myelitis.

Adrenoleukodystrophy in France: an epidemiological study.

An epidemiological study was conducted in France to estimate the prevalence of adrenoleukodystrophy, a severe neurologic X-linked disorder affecting boys and young men. 129 cases were collected. Analysis of all cases born between 1956 and 1986 with available clinical history allowed the calculation of the lowest estimation of the prevalence: 1 in 100,000 male births.

[Transient global amnesia]. / L'ictus amnésique.

Transient global amnesia is a momentary neurological accident frequently encountered in subjects over 50 years of age. Its diagnosis is purely clinical and rests on the sudden occurrence of retention amnesia associated with retrograde amnesia without disturbances in speech or other neurological deficits. The amnesia totally regresses within less than 24 hours. Paraclinical examinations add nothing to the diagnosis. The cause of transient global amnesia is unknown, and the various hypotheses that have been put forward (e.g. epilepsy, transient ischaemia or migraine) have not been confirmed by clinical and epidemiological studies. Despite a low, but real risk of recurrence, the prognosis is perfectly benign, and there is no need for curative or preventive treatment.

[Neurologic complications of cocaine abuse]. / Accidents neurologiques liés à l'usage de la cocaïne.

Cocaine is increasingly used by drug addicts. It is considered harmless, but numerous, varied and often serious complications due to its abuse have been published. Among these, neurological complications are in the forefront. They include generalized or partial epileptic seizures, ischaemic or haemorrhagic cerebral vascular accidents, visual loss caused by optic neuropathy or by retinal artery occlusion, headaches and exacerbation of tics. Infections of the central nervous system are possible via endocarditis or septicaemia of venous or nasal origin. Neurological disorders may also occur as a consequence of a major cardiovascular complication induced by cocaine (myocardial infarction and/or dysrhythmia, aortic dissection). These neurological complications are unpredictable, and they weigh heavily on the functional and sometimes vital prognosis in habitual or occasional cocaine abusers.

[Lesions of the brain stem and cerebellum of alcoholic and nutritional deficiency origin]. / Atteintes du tronc cérébral et du cervelet d'origine alcoolique et carentielle.

Lesions of the brain stem and cerebellum due to toxic substances or nutritional deficiencies are mostly seen in chronic alcohol abuse and, more rarely, in severe malnutrition. Four clinical entities are described: Wernicke's encephalopathy is the most frequent of these, with progressive development of disorders of consciousness, oculomotor palsies and ataxia. The condition regresses under massive vitamin BA therapy, but it often leaves neuropsychological sequelae (e. g. Korsakoff's syndrome). The best treatment is prevention by giving thiamine to alcoholics and to malnourished subjects who receive glucose infusions. Cerebellar atrophy of alcoholic origin is responsible for subacute ataxia which, as a rule, is definitive. Central pontine myelinolysis is rare and presents as sudden quadriplegia with pseudobulbar palsy and sometimes "locked-in" syndrome. Beside alcohol, a frequent cause is major ionic disorders, such as hyponatraemia or its excessively rapid correction. Pellagra is a classical disease rarely seen in this country. It is due to nicotinamide (vitamin PP) deficiency.

[Peroxisomes and neurologic diseases]. / Peroxysomes et pathologie neurologique.

Peroxisomes are ubiquitous subcellular organelles varying in number, size and enzymatic content according to species, tissues or physiological states. Microperoxisomes are present in the central nervous system and in muscle. Peroxisomes participate in anabolic and catabolic processes, including ether-lipid synthesis, bêta-oxidation, bile acid synthesis, prostaglandin catabolism. Very long chain fatty acids are specific substrates of peroxisomal acyl-CoA oxidase. Peroxisomal disorders occur as two main groups: 1/ disorders with multiple deficiencies of peroxisomal functions: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, rhizomelic chondrodysplasia punctata; 2/ disorders with a single peroxisomal enzyme defect: X-linked adrenoleukodystrophy, acatalasemia, type 1 hyperoxaluria, pseudo-Zellweger syndrome. Present therapy is tentative with some limited success. It includes peroxisomal inductors and lipid-controlled diet. Prenatal diagnosis and heterozygote detection allow genetic counselling in some peroxisomal disorders.

[Aortic aneurysm with vena cava thrombosis occurring in Behcet's disease]. / Anévrisme aortique avec thrombose cave contiguë au cours d'une maladie de Behcet.

We report a case of Behcet's disease complicated by aortic aneurysm and contiguous vena cava thrombosis due to compression. Arterial aneurysms are uncommon in the course of Behcet's disease and are associated with a poor prognosis owing to the risk of rupture. Vena cava thrombosis is found in 10% of cases; pulmonary embolism is infrequent. Venous and arterial lesions usually evolve independently. In most cases they are consecutive to vasculitis. The case reported herein is uncommon because of simultaneous and contiguous venous and arterial lesions. Eighteen months after aorto bi-iliac graft and inferior vena cava ligature, there is no recurrence of thrombosis nor aneurysm with a treatment including heparin, colchicine and azathioprine.

[Sleep and dreams in Korsakoff's amnesia due to alcoholism]. / Sommeil et rêves dans l'amnésie de Korsakoff d'origine alcoolique.

Sleep and dreams in 15 chronic alcoholic patients with amnesia were compared with sleep and dreams of 15 age- and sex-adjusted normal subjects. The patients were subjected to psychological tests in order to determine their I.Q. and their memory disturbances. All subjects had two nights of polygraphic recordings; the first tested the natural sleep organization. During the second night, they were awakened 7 min after the onset of each REM sleep episode, and, at least once, 20 min after the onset of a stage II episode, in order to record on a tape their dream reports according to a standardized protocol. The sleep patterns of the amnesic patients did not show any significant alteration. However, after wakening during the night, patients exhibited a higher tendency to return to REMS than controls. There was still some dream activity in those patients, although noticeably less frequently, and their dream activity had a very poor verbal expression. However, there was no change with respect to the spatio-temporal organization, sensorial perceptions, motor activity and verbalizations during their dreams.

Adult adrenoleukodystrophy: a sporadic case?

This is a report of a case of the adult cerebral form of X-linked ALD. The 27-year-old patient presented with psychiatric disturbances. NMR was performed and compared to CT scan to define cerebral demyelination. The level of hexacosanoate was found to be increased in the patient's serum. Biochemical analysis of the patient's mother's serum and cultured fibroblasts and of serum samples from 10 other members of the family who could have been carriers of this X-linked disease, produced negative results. Hence, it is most likely that this case has occurred sporadically. HLA determination revealed the DR2 antigen which is often associated with multiple sclerosis.

[Adult disclosure of a case of familial adrenoleukodystrophy]. / Révélation à l'age adulte d'un cas d'adrénoleucodystrophie familiale.

We report a case of adult adrenoleukodystrophy. The patient, originating from North Africa, had no clinical history until age 23. The first disorder, a spastic paraparesis, occurred after a 5 days coma following a cranial traumatism. The course of the disease was then progressive remitting and the patient died at age 30. Total duration was 7 years. The diagnosis of A.L.D. was ascertained by the determination of excessive very long chain fatty acid level in plasma. Endocrinological study revealed adrenal insufficiency but darkening of the skin was masked by racial pigmentation. The patient was the oldest of 10 children. 3 of the brothers died of childhood A.L.D. around the age of 12 after a progressive 2 years course. The mother and 2 asymptomatic brothers have increased plasma levels of hexacosanoic acid and A.C.T.H. The particularity of this adult case is the lack of spinal demyelination. Pathological studies revealed a widespread cerebral demyelination with an inflammatory process. The features of this case are compared to those of the 13 other reported cases of adult A.L.D.