Troubles psychotiques révélateurs de maladie de Creutzfeldt-Jakob sporadique

The onset of the sporadic Creutzfeldt-Jakob disease is usually characterized by dementia or neurological symptoms. We are reporting the case of a 31 year-old man with a sporadic Creutzfeldt-Jakob disease characterized by a schizophrenia-like onset. The schizophrenic symptoms developed for sixteen months before the neurological symptoms appeared. The psychiatric onset of the present case was linked with the early age of onset and the long duration of the disease and could have been influenced by polymorphism of the codon 129 of the prion protein gene (PRNP) and a type of pathological prion protein (PrPres).     

Maxillary sinus puncture and culture in the diagnosis of acute rhinosinusitis: the case for pursuing alternative culture methods.

OBJECTIVE: Traditional assessments of the microbial flora associated with acute bacterial rhinosinusitis have relied on maxillary sinus punctures (taps) and culture. These taps are now considered the gold standard for obtaining cultures and are used as the method of identifying bacterial pathogens in antimicrobial trials. Maxillary sinus taps are limited by discomfort to the patients and technical concerns. Because of these factors, the standard of performing taps has limited antibiotic trials and microbial surveillance. Alternatives to maxillary sinus taps have been explored. STUDY DESIGN: We conducted a retrospective, systematic review of the literature from 1950 to 2000 of articles comparing culture techniques in the nose and paranasal sinuses for acute bacterial rhinosinusitis. RESULTS: Nasal cultures have poor correlation to maxillary sinus cultures, whereas there is 60% to 85% concordance between endoscopically guided middle meatal cultures and maxillary sinus cultures. These studies, however, are all limited by small sample sizes and therefore are inadequate to make any concrete recommendations regarding the relative role of endoscopically guided middle meatal cultures as a formal method of pathogen identification in acute bacterial rhinosinusitis. CONCLUSION: A formal prospective study with sufficient sample size to assess the concordance between the microbial flora of the maxillary sinus punctures and middle meatal cultures in acute rhinosinusitis is recommended.     

Changes in the Eeg Background Activity of Children with Acute Lymphoblastic Leukemia During Cytotoxic Therapy

Thirteen children with acute lymphoblastic leukemia (ALL) were investigated before and during cytotoxic therapy. EEG findings were correlated with the clinical course and the therapy protocol and compared with normal data obtained from 295 healthy children. Frequency analysis of the background activity of the EEG revealed an initial slowing of the background activity prior to therapy and further slowing each time a combination of vincristine (VCR), daunorubicine (DAU) or adriblastine (ADR), prednisone (FRED), and L-asparaginase (L-ASP) was administered. The slowing of the background activity correlated only with the administration of these drugs. DAU, ADR, and FRED are not known to influence the EEG; therefore, VCR and L-ASP remain the primary candidates responsible for the central nervous system alteration.     

Histiocytic necrotizing lymphadenitis (Kikuchi's disease)

Histiocytic necrotizing lymphadenitis (Kikuchi's disease) is a benign disorder predominantly of young women who present with cervical adenopathy. It has, on occasion, been misdiagnosed as a malignant neoplasm. Little mention of this condition has appeared in the otolaryngologic literature. A patient with Kikuchi's disease is reported and the pertinent literature is reviewed. Because both the clinical and pathological aspects of Kikuchi's disease are important in establishing the diagnosis, effective consultation between the head and neck surgeon and the pathologist is crucial.     

Magnetic resonance imaging in full-term infants with repetitive focal seizures.

We report two full-term infants who developed repetitive focal seizures within the first 48 hours of life. Neither infant had predisposing factors and there were no abnormalities on a computed tomography (CT) scan performed on day 2 of life. Magnetic resonance imaging (MRI) performed during the second week of life showed a focal hemorrhagic infarction in both patients. We conclude that either an MRI or a contrast-enhanced CT scan should be obtained within 1 week in patients in whom the initial imaging technique failed to reveal a focal lesion, at which time a cerebral infarction can be diagnosed with greater sensitivity.     

Medical liaisons for continuity of head and neck cancer care.

The role of assistance in coordinating treatment and follow-up on the effectiveness and reliability of long-term care of head and neck cancer patients was evaluated. A comparison between the Department of Radiation Oncology, where a liaison team assists cancer patients, and the Department of Otolaryngology, where there were no such personnel, was made. Of 124 consecutive patients with head and neck squamous cell carcinoma, 31 were treated with surgery alone and 93 had radiation therapy alone or in combination with surgery. Patients were nearly equally divided among stages, although a higher percentage receiving surgery were stage I and those receiving radiation were stage IV. Nineteen radiation patients and 1 surgery patient have died; this is consistent with the differences in stage distribution. Thirteen of 31 surgery patients had unreliable follow-up, with 7 lost to follow-up. Only 8 of 93 radiation patients were unreliable, with 4 lost. The difference is statistically significant. The importance of nursing/social work liaisons in coordinating treatment and follow-up of head and neck cancer patients is discussed.     

Genome-wide linkage survey for genetic loci that affect the risk of suicide attempts in families with recurrent, early-onset, major depression.

We previously described the results of a genome-wide linkage survey for genetic loci that influenced the development of unipolar mood disorders in 81 families identified by individuals with Recurrent, Early-Onset, Major Depressive Disorder (RE-MDD) [Zubenko et al. 2003b; Am J Med Genet (Neuropsychiatr Genet) 123B:1-18]. In the current study, we extended this linkage analysis by including the history of a suicide attempt as a covariate to identify chromosomal regions that harbor genes that influence the risk of this behavior in the context of mood disorders. This approach identified six linkage peaks with maximum multipoint DeltaLOD scores that reached genome-wide adjusted levels of significance (2p, 5q, 6q, 8p, 11q, and Xq). Four of these (2p, 6q, 8p, and Xq) exceeded the criterion for "highly-significant linkage" (genome-wide adjusted P < 0.001) recommended by Lander and Kruglyak [1995; Nat Genet 11:241-246]. The strongest evidence for linkage was observed in analyses employing affected relative pairs (ARPs) with the most severe and disabling Mood Disorders: Depression Spectrum Disorder and RE-MDD. The highest DeltaLOD score that emerged from this linkage analysis, 5.08, occurred for ARPs with Depression Spectrum Disorder at D8S1145 (37.0 cM, 18.2 Mbps, P < 0.0001) at cytogenetic location 8p22-p21. Significant linkage results on Xq arose from analyses of ARPs with RE-MDD at DXS1047 (143 cM, 127.8 Mbps, DeltaLOD = 3.87, P < 0.0001), a finding that may contribute to the higher rate of suicide attempts among women than men. These findings provide evidence for suicide risk loci that are independent of susceptibility loci for Mood Disorders, and suggest that the capacity for suicide risk loci to affect the development of suicidal behavior depends on the psychiatric disorder or subtype with which they interact.     

Ultrastructural Changes Associated with Peripheral Neuropathy in HIV/AIDS

Light and electron microscopic studies were performed on neuromuscular biopsy specimens from 12 neurologically affected seropositive patients, 7 with the acquired immune deficiency syndrome (AIDS), 2 with AIDS-related complex, and 3 with no symptoms except for neuropathy. All patients had an axonal injury associated or unassociated with demyelination and peripheral neurogenic atrophy. Capillary lesions were consistently present, which seems to be a new finding. Moreover, tubuloreticular inclusions (TRIs) were found in endomysial (9 of 12 cases) and endoneurial (7 of 12 cases) vessels. Statistical analysis showed that TRIs in more than 20% of endomysial vessels correlated with a survival time shorter than 12 months (P = 0.028). Thus the quantification of TRIs seems to be one of the vital prognostic elements in this patient population.