A case of extrahepatic portal hypertension

Bleeding from the proximal part of the gastrointestinal (GI) tract is not uncommon in children. Children could present with anemia secondary to chronic occult bleeding in the gastrointestinal tract or as anemia secondary to acute exsanguinations and can present in hypovolemic shock. There are various causes of upper GI bleeding in children. A systematic approach in evaluating these children is essential so that the diagnosis is made in timely manner and appropriate management is begun early.     

Low-grade adenocarcinoma of endolymphatic sac origin

We present a case of papillary cystic low-grade adenocarcinoma of endolymphatic sac origin. These tumors are very rare and only a few cases have been reported in literature. They have a protracted clinical course causing progressive invasion and bony destruction. Radiologically, they appear as a heterogenous lobulated vascular mass, invading bone and compressing surrounding structures. Histologically, these tumors are composed of uniform population of cells, resembling normal endolymphatic sac epithelium. This causes considerable diagnostic difficulty. A strong index of suspicion along with clinical and radiological correlation is essential to arrive at a correct diagnosis. In some bilateral cases, association with von Hippel-Lindau disease has been noted. Radical mastoidectomy and temporal bone resection, which may sometimes necessitate sacrifice of cranial nerves, is the treatment of choice.     

Neurological disorders in children and adolescents

Objective  To ascertain the prevalence of active epilepsy, febrile seizures (FS), cerebral palsy (CP) and tic disorders (TD) in aged 19 years or less. Methods  This was a cross-sectional observational study conducted as a two-stage door-to-door survey of a stratified randomly selected population in 2003-04. Trained field workers screened the population followed by case examination by the field neurologist. Results  A total of 16979 (male 8898, female 8081) subjects aged ≤ 19 years were surveyed. The prevalence rates per 100,000 population of active epilepsy, FS, CP and TD with 95% confidence intervals are 700.87 (580.60–838.68), 1113.14 (960.07–1283.59), 282.70 (CI 208.43–374.82) and 35.34 (12.96–76.92) respectively. Active epilepsy prevalence shows a rising trend and that of other disorders a declining trend with age. Of the epileptics who had brain CT scans, 23.4% showed single or multiple lesions suggestive of neurocysticercosis. Regarding treatment, 23.5% of the epileptics never received any antiepileptic drugs. Among those with history of FS, 9.5% developed epilepsy later on. The prevalence of FS among slum dwellers is lower than in the non-slum population. Among CP cases, 39.6% gave history of birth anoxia, 16.7% kernicterus and 31.3% epilepsy. Prevalence of CP is significantly associated with lower education status. Conclusion  The prevalence of CP and TD is lower than reported from western countries. CP prevalence is also comparatively lower than in many community studies from India. Compared to western nations, higher proportion of FS cases develops epilepsy. A third of the CP cases have seizures which is higher than in many Indian studies. Birth anoxia is a common cause of CP and educational underachievement is frequent.     

Auditory efferent function is affected in multiple sclerosis

OBJECTIVE: This study evaluated efferent medial olivocochlear (MOC) function in patients with multiple sclerosis (MS). Various afferent auditory abnormalities have been described in MS, but there is a paucity of data on efferent function. The brain stem is a site of predilection for MS plaques and the efferent MOC pathway may be affected at this level. METHODS: The study included 30 patients who had normal hearing. According to MRI findings, they were divided into two groups: those with an identifiable brain stem lesion (n = 10) and those with MS lesions in other parts of the central nervous system but without demonstrable MS plaques in the brain stem (n = 20). MOC function was evaluated by the olivocochlear suppression test, using transient evoked otoacoustic emissions. All subjects underwent standard auditory tests, including pure-tone audiometry and recording of auditory brain stem evoked responses. Twenty-two healthy subjects with normal hearing, matched for age and gender, served as a reference group for the auditory data. RESULTS: The results showed that 66.6% of all patients had reduced MOC function, particularly those (90%) with identified lesions of the brain stem on MRI. Furthermore, abnormal MOC function was found in 55% patients without evidence of a brain stem lesion on MRI. CONCLUSIONS: This study provides the evidence for a deficit of efferent auditory function in the majority of patients with MS. Taking into consideration the possible roles of the MOC system in processing of auditory information, abnormal MOC suppression in patients with MS may explain a variety of auditory presentations that are currently largely overlooked. This study also highlights the diagnostic value of the MOC suppression test as a site-of-lesion diagnostic test in MS and in identifying subtle brain stem lesions undetected by MRI, suggesting that subtle brain stem lesions may exist and that the MOC suppression test is sufficiently sensitive to detect them. Accordingly, the MOC suppression test may provide a tool for an early diagnosis of MS.