Poliomyelitis-like syndrome following asthmatic attack (Hopkins' syndrome)--recovery associated with i.v. gamma globulin treatment.

A report on a 15-year-old male with a diagnosis of poliomyelitis-like syndrome (Hopkins' syndrome) following an asthmatic attack is presented. The prognosis of Hopkins' syndrome is usually poor and the patients remain with permanent paralysis of the affected limb. The outcome correlates with severity of the initial injury to the anterior horn cell as reflected by abnormal electrophysiologic studies. This is the first case report of treatment with i.v. gamma globulin in Hopkins' syndrome which resulted in a nearly complete recovery.

MELAS syndrome: peripheral neuropathy and cytochrome C-oxidase deficiency: a case report and review of the literature.

A 4-year-old boy presented with developmental delay, aggressive behavior, and incoordination. His EEG showed a diffuse encephalopathy. At age 10 he developed convulsions and severe migraine-like headaches. Muscle wasting, arreflexia, and lactic acidemia following exercise were noted. Electromyography was myopathic and nerve conduction studies revealed a peripheral neuropathy. Muscle biopsy demonstrated variation in fiber size and an excess of lipid droplets. He than had several stroke-like episodes and periods of unconsciousness, associated with severe metabolic acidosis. Muscle cytochrome C oxidase was abnormally low. This boy displayed the classical clinical and biochemical features of MELAS syndrome, namely Mitochondrial myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes. Treatment included carnitine, vitamin C, vitamin K, riboflavin, coenzyme Q10, and corticosteroids. He died at the age of 14 years following an episode of seizures, coma, and gastrointestinal hemorrhage. This is the first reported case of MELAS syndrome in Israel.

Melkersson-Rosenthal syndrome.

Four patients with recurrent upper lip and facial swelling and lingua plicata together with peripheral facial nerve palsy involvement were diagnosed as having Melkersson-Rosenthal syndrome. Because of the rarity of this syndrome in children, we present the results of follow-up evaluations of our patients and review the literature.

Swimming and grommets.

BACKGROUND: Traditionally, children with tympanostomy ventilating tubes, or grommets, were advised that water should not enter their ears in order to prevent ear infections. This group of children has been considered somewhat handicapped regarding swimming. We conducted a prospective study to determine if there is a relation between suppurative otitis media and surface swimming in children with grommets. METHODS: Forty-two children with tympanostomy ventilating tubes were included in this study. Of the 42 children, 22 were swimmers and 20 were nonswimmers, who served as the control group. The age range was 3 to 12 years, and there was no difference in the age distribution between the groups. Surface swimming was allowed without earplugs or a bathing cap, although it was mandatory to use polymyxin B-neomycin-hydrocortisone eardrops at bedtime on the day of swimming. No diving was allowed. RESULTS: Three of 22 swimmers and 2 of 20 nonswimmers developed otorrhea. In 4 of the 5 children, the otorrhea was followed by an upper respiratory tract infection. In all cases, a bacterial culture revealed Pseudomonas. The ear drainage was easily controlled with local otic treatment in all the patients. CONCLUSIONS: Taking into consideration the possible risks of infection and bearing in mind the value and joy of swimming to children and parents, families should be reassured that surface swimming does not increase the risk of infection in children with tympanostomy tubes.

Benign paroxysmal torticollis in infancy.

Benign paroxysmal torticollis in infancy is characterized by periods of torticollic posturing of the head. The onset of the episodes usually occurs during the first month of life and may recur at varying intervals until the age of 1-5 years. This appears to be a self-limited disorder. The follow-up of 7 patients with benign paroxysmal torticollis is presented.

Extraordinary daytime urinary frequency in children.

BACKGROUND: Sudden onset of daytime urinary frequency of a small amount of urine in a previously toilet-trained child can be a disturbing problem. Psychosocial problems, problems at school, or problems within the family have to be taken into consideration. METHODS: Fifteen children with sudden onset of isolated daytime urinary frequency were evaluated and followed for a period of 12 to 18 months. A thorough medical and family history was obtained for each child, with special attention given to any psychosocial problems the child had experienced. All the children underwent a physical examination, complete urinalysis, and ultrasonography of the kidneys and bladder. RESULTS: In all of the patients, the urinalysis and ultrasonographic findings were within normal limits. A trigger factor was identified as the cause of urinary frequency in each case. CONCLUSIONS: Because urinary frequency is usually a benign self-limited condition, an extensive urological evaluation is not indicated. In most cases, providing reassurance to the parents and the child is the only intervention necessary.

Postcircumcision urinary tract infection.

The possible association of urinary tract infection (UTI) with ritual circumcision on the eighth day of life was studied by analyzing the epidemiology of urinary tract infections during the first year of life in 169 children with UTI (56 males and 113 females) born in Israel from 1979 to 1984. Forty-eight percent of the episodes of UTI occurring in males appeared during the 12 days following circumcision, and the increased incidence during that period was highly significant. The median age of the males at the time of the UTI was 16 days, compared with seven months in females. Ritual Jewish circumcision as practiced in Israel may be a predisposing factor for UTI during the 12-day period following that procedure.

Cerebellopontine angle lipoma in a teenager.

Lipomas of the cerebellopontine angle are very rare lesions. To date, 18 patients have been reported, 17 of whom were adults. A second child is described with cerebellopontine angle lipoma.