Study of Normal Flora in the Pharynx of Healthy Children.

To improve our current understanding of normal flora in children, we investigated bacterial isolates from the pharynx and nasopharynx of 173 and 233 healthy children, respectively. The bacterial isolation rates were compared among three age groups: infants (<1 year), toddlers (1-5 years), and school-aged children (6-15 years). Gram-positive cocci were the predominant bacteria in the pharynx (Streptococcus mitis/oralis, 87.3%; Streptococcus salivarius, 54.3%; Rothia mucilaginosa, 41.6%; Staphylococcus aureus, 39.3%). Among infants, S. salivarius and Neisseria subflava, which are related to the development of teeth, were significantly lower than in the other age groups (P <0.0001, S. salivarius; P <0.01, N. subflava). With the exception of Corynebacterium pseudodiphtheriticum (44.2%, gram-positive rods), gram-negative rods largely predominated the nasopharynx (Moraxella catarrhalis, 32.1%; Moraxella nonliquefaciens, 28.3%). Among toddlers, M. catarrhalis and Streptococcus pneumoniae, which are the most common pathogens in acute otitis media, were significantly higher than in the infant group (P <0.05). Among the bacterial species implicated in pediatric respiratory infections, Streptococcus pyogenes was isolated in 3.5% of the pharyngeal samples. S. pneumoniae and Haemophilus influenzae were isolated in 22.3% and 17.2% of the nasopharyngeal samples, respectively. In conclusion, the normal flora of the respiratory tract differs not only by the sampling site but also by the age group.

Negative scotopic ERG and photopic ERG ON response impairment in a patient with normal dark adaptation.

PURPOSE: To present ocular findings in a patient who showed negative scotopic electroretinogram (ERG) and reduced ON response, but normal dark adaptation. CASE: An 18-year-old Japanese male patient who complained of severe asthenopia. His corrected visual acuities were 1.2 in both eyes. His fundi were normal. He had normal contrast sensitivity and normal dark adaptation. METHODS: The patients underwent ERG (including the standard protocol and photopic long flash recordings). RESULTS: The amplitudes of the rod ERG b-wave were reduced. The scotopic standard combined ERG response showed negative configuration. The photopic response to long flash revealed the reduced b-wave (ON response), while the amplitude of the first peak of the d-wave (OFF response) was within the normal range. CONCLUSIONS: Postsynaptic abnormalities in both the rod and cone ON-pathways, which are often found in patients with night blindness, were suggested in the ERG findings, but the dark adaptation of our patient was normal. Neuromuscular evaluation of the patient and ophthalmological evaluation, including ERG, of his parents were normal. To our knowledge, the ophthalmological and electrophysiological findings of our patient cannot be attributed to any known clinical entity.

Familial amyotrophic lateral sclerosis with bulbar onset and a novel Asp101Tyr Cu/Zn superoxide dismutase gene mutation.

We describe a patient with familial amyotrophic lateral sclerosis (FALS) in whom we identified a novel missense mutation in exon 4 (Asp101Tyr) of the Cu/Zn superoxide dismutase (SOD1) gene. The disease started with a bulbar symptom (rapidly progressive hoarseness) and at autopsy showed degenerative changes restricted to the upper and lower motor neuron systems (more strictly, with lower motor predominance, showing the most severe degeneration in the nucleus ambiguus). Occasional intracytoplasmic Lewy-body-like hyaline inclusions that were immunoreactive for ubiquitin and SOD1, but immunonegative for neurofilament protein, were found in the lower motor neurons. This is the first report of hoarseness as the initial manifestation of FALS. This SOD1 gene mutation may be associated with a particular clinicopathological phenotype.

[A case of polymyalgia rheumatica with improved steroid-responsibility after discontinuing carbamazepine].

A 70-year-old woman under anticonvulsive therapy with carbamazepine and valproate was diagnosed as polymyalgia rheumatica. She responded to the prednisolone therapy so poorly that she required high dose prednisolone and methotrexate, and it was difficult to reduce prednisolone. After discontinuing of carbamazepine, her steroid response improved immediately. Carbamazepine is known to induce hepatic enzyme CYP3A4 and alter metabolism of other drugs. In our case, the effect of prednisolone might have been reduced by carbamazepine. This case suggests more attention should be paid to the interaction between carbamazepine and prednisolone.

[Steroid-pulse therapy in Guillain-Barré syndrome associated with cytomegalovirus infection: a case report].

We report a 27-year-old man with Guillain-Barré syndrome (GBS) preceded by cytomegalovirus infection. He was admitted to our hospital because of distal dominant weakness and sensory disturbance 5 days after fever. Double filtration plasmapheresis (DFPP) was performed and clinical symptoms temporary but dramatically improved. However facial nerve palsy, difficulty in swallowing food, weakness, dysautonomia and respiratory failure rapidly progressed within 5 days after the onset. Repeated DFPP failed to improve his symptoms. Two months after the onset, he did not improve at all. On T1-weighted MRI, nerve roots were still enhanced with gadolinium, and CSF examination revealed 1,324 mg/dl of protein. These findings suggested us the existence of continuous inflammation on nerve roots. We gave steroid-pulse therapy. He dramatically improved after this treatment. We repeated steroid-pulse therapy for seven times. He was discharged without any major complication 6 months after the onset. Steroid-pulse therapy should be considered in GBS patients associated with CMV infection when other conventional treatments are ineffective.