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Presents a letter to the editors responding to the essay "A Modest Proposal," by Peter Steams, published in the May 2005 issue.

A letter to the editor is presented on the author's experiences with the publication "Inuit Art."

The presenilin-1 (PS1) gene mutation (Pro117Leu), recently identified in a Polish family is characterized by the earliest reported onset (from 24–31 years) of Alzheimer disease (AD) and a very short duration of disease (4–6 years). The neuropathology of 2 subjects with this PS1 mutation (ages at death: 35 and 37 years) was compared to four Down syndrome (DS) patients (mean age at death: 62 years) and 4 sporadic AD patients (mean age at death: 79 years with a mean duration of disease of 18 years). The Polish familial AD (FAD) patients showed a marked increase in the amyloid burden of 2–6-fold in most areas of the brain. The entorhinal cortex was an exception where the amyloid burden was similar in each category of patient. Some brain regions of the Polish FAD patients showed a massive increase of amyloid, such as the molecular layer of the cerebellum where a 7- and 25-fold increase was noted, compared with DS and sporadic AD patients respectively. The cerebellar vessel amyloid burden was also greatly increased in the FAD patients, reflecting a vascular compartment specific increase of amyloid B deposition. The presence of this PS1 mutation has an even greater effect on both vascular and parenchymal amyloid deposition, than the overexpression of the amyloid β precursor protein present in DS patients, suggesting that PS mutations can be a critical factor determining amyloid deposition.

Proinflammatory cytokines (interleukin [IL]-1β, tumor necrosis factor [TNF]-α, and IL-6) and soluble intercellular adhesion molecule-l (sICAM-1) were measured in paired cerebrospinal fluid (CSF) and serum samples from patients with subacute sclerosing panencephalitis (SSPE), multiple sclerosis (MS), or other neurologic diseases(OND) by ELISA. IL-1β was significantly increased in CSF of the SSPE group compared with levels in the MS or OND group. IL-1β CSF/serum ratios were higher in the SSPE than in the MS or OND group. TNF-α and IL-6 levels were similar in the 3 groups. CSF sICAM-1 was higher in the SSPE group than in the MSor OND group. sICAM-1 CSF/serum ratios were higher in the SSPE than the OND group. The increased CSF/serum ratios of IL-1β and sICAM-1 in SSPE indicate synthesis of IL-1β and sICAM-1 in the central nervous system and may be important in the pathogenesis of disease.

REPLY We were very interested in Dr. Taylor's and Miss Siegel's observation resulting from their five-month study of 23 children with cystic fibrosis (C/F) wherein they found normal hearing. The hearing study on C/F patients at the Children's Hospital of the DC was conducted for over four years; and on the average each C/F patient was followed for over three years. Initially, we were surprised by the results of mild hearing loss in 27% of the tested C/F patients. Consequently, the study was repeated, embracing 70 C/F patients among whom 26% were found to have mild transient hearing losses due to conductive pathology. This second study is to be reported in an upcoming 1970 issue of the journal, Clinical Pediatrics.We completely agree with Dr. Taylor's and Miss Siegel's opinions that C/F is an hereditary disease and as such should have a uniform expression. However, since the disease has been