Relationship between daily swallowing frequency and pneumonia in patients with severe cerebral palsy.

BACKGROUND: Aspiration pneumonia is a major complication that occurs in patients with severe cerebral palsy and is associated with their survival prognosis, necessitating appropriate assessment and response. We focused on swallowing frequency as an index of daily swallowing function due to the difficulty in evaluating the risk of pneumonia. The swallowing motion protects the airway by safely directing the food, saliva, and secretions accumulated in the pharynx into the esophagus to prevent aspiration and entry into the trachea. Thus, swallowing frequency may be correlated with the incidence of pneumonia. In this study, we aimed to investigate the relationship between swallowing frequency and history of pneumonia in patients with severe cerebral palsy. METHODS: Fifty-seven patients with cerebral palsy were included in this study. Swallowing frequency was measured three times for each patient on separate days, and the reproducibility was examined by calculating the intraclass correlation coefficient. Further, the relationship between swallowing frequency and history of pneumonia was investigated using multivariate logistic regression analysis. RESULTS: While swallowing frequency differed between participants, it was constant within individuals (intraclass correlation coefficient: 0.941). Furthermore, the swallowing frequencies per hour were 12.2 ± 12.2 and 27.0 ± 20.4 in the patient groups with and without a history of pneumonia, respectively (P < 0.001). Swallowing frequency (odds ratio: 10.489, 95% confidence interval: 2.706-40.663, P = 0.001) was significantly associated with the incidence of pneumonia in the previous year. CONCLUSIONS: Swallowing frequency could be used as an index for assessing the risk of dysphagia and pneumonia in patients with severe cerebral palsy.

Twelve-year-old girl with intracranial epidural abscess and sphenoiditis.

We report the case of a 12-year-old girl with an intracranial epidural abscess and sphenoiditis. Although she had no history of sinusitis, she developed acute severe headache, fever, and vomiting. Emergent CT and MRI showed a spherical space-occupying lesion of diameter 3 cm in the right cranial fossa with rim enhancement. The lesion was thought to be an epidural abscess adjacent to the right sphenoiditis. On the basis of the MRI findings, we performed emergent surgery to drain the abscess and sinusitis because of severe and rapidly worsening headaches. The patient showed great improvement the day after the operation. Intravenous antibiotics were administered for 8 days. She has completely recovered, with neither sequelae nor recurrence at 7 months after the operation. We believe that this report will be a useful reference for cases of acute onset headache and may be helpful in diagnosis and treatment decisions for severe sinusitis-related intracranial abscess in childhood.

A case of TUBA1A mutation presenting with lissencephaly and Hirschsprung disease.

Gene mutation of tubulin alpha-1A (TUBA1A), a critical component of microtubules of the cytoskeleton, impairs neural migration and causes lissencephaly (LIS). The approximately 45 cases of disease-associated TUBA1A mutations reported to date demonstrate a wide spectrum of phenotypes. Here we describe an 8-year-old girl with lissencephaly, microcephaly, and early-onset epileptic seizures associated with a novel mutation in the TUBA1A gene. The patient developed Hirschsprung disease and the syndrome of inappropriate antidiuretic hormone secretion (SIADH), which had not previously been described in TUBA1A mutation-associated disease. Our case provides new insight into the wide spectrum of disease phenotypes associated with TUBA1A mutation.

[A case of respiratory bronchiolitis-associated interstitial lung disease].

A 37-year-old man was referred to our hospital with a cough. His blood test result and chest radiography findings were normal. High resolution chest computed tomography (HRCT) showed diffuse ground glass attenuation (GGA) with centrilobular distribution in the bilateral upper lung fields. As diagnosis could not be made using transbronchial lung biopsy and bronchoalveolar lavage, video-assisted lung biopsy was performed. The histological findings were a thickening of the alveolar walls with infiltration of lymphocyte-dominant inflammatory cells, and exudation of pigmented macrophages in the air spaces of the respiratory bronchioles and alveolis. We diagnosed his illness as respiratory bronchiolitis-associated interstitial lung disease. Six months after stopping smoking, his symptoms, and the GGA on chest HRCT improved.

[A case of nonspecific interstitial pneumonia with reversed halo sign on chest HRCT].

A 39-year-old man was referred to our hospital with anterior chest discomfort, dry cough and shortness of breath. His blood test revealed mild inflammatory change and high serum KL-6 levels. Chest radiograph and computed tomography (CT) showed ground glass attenuation with volume loss in both lower lung fields, and in particular a reversed halo sign was shown on high-resolution CT (HRCT). As transbronchial lung biopsy and bronchoalveolar lavage did not enable a diagnosis, video-assisted thoracic surgery was performed. The histological findings of the resected specimen showed cellular nonspecific interstitial pneumonia. This suggested the possibility of collagen vascular disorder (CVD) associated with interstitial pneumonitis, but no criteria of CVD were fulfilled. Although the reversed halo sign is relatively specific for cryptogenic organizing pneumonia, we report a case of cellular nonspecific interstitial pneumonia showing this sign on chest HRCT.

[A child with non-herpetic acute limbic encephalitis affecting the claustrum and hippocampus].

We experienced an 8-year-old-boy with non-herpetic acute limbic encephalitis (NHALE), who developed headache, convulsion, consciousness disturbance, and ataxia following cold like symptoms. Disturbance of short term memory and a change of character were recognized. Myoclonic seizures and generalized tonic clonic convulsions developed, that responded to antiepileptic agents. Although other symptoms resolved spontaneously, short term memory disturbance persisted. Brain MRI demonstrated the lesion involving the bilateral claustrum and right hippocampus. Three months later, the lesion in the claustrum disappeared, but the hippocampus still showed slight hyperintensity on FLAIR image of MRI. Autoantibodies against glutamine receptor were detected in the cerebrospinal fluid and plasma, which suggested the involvement of immunologic disturbances in this disease. In NHALE, many cases have been reported in adults but not in children, and the further attentions should be paid to childhood-onset NHALE.

Attenuation of biologic compensatory action of cardiac natriuretic peptide system with aging.

Although plasma B-type natriuretic peptide (BNP) levels increase with age, the mechanisms responsible for this increase are unknown. We investigated the predictors of elevated BNP in older subjects without cardiac systolic dysfunction and overt renal dysfunction. Furthermore, we analyzed the relations between BNP and its second messenger, cyclic guanosine monophosphate (cGMP), to aging. In 252 subjects (mean age 69 +/- 12 years) with left ventricular ejection fraction >/=50% and creatinine levels <==1.5 mg/dl, plasma levels of BNP, cGMP, blood urea nitrogen, creatinine, and beta2-microglobulin (an endogenous marker of renal function), estimated glomerular filtration rate, and echocardiographic data were prospectively evaluated. Plasma BNP levels increased with age (r = 0.4, p <0.0001). With use of multivariate analysis, predictors of elevated BNP levels were age, use of beta blockers, and serum beta2-microglobulin levels. The molar ratio of cGMP to BNP significantly decreased with aging (r = 0.55, p <0.0001). Elevated BNP in older subjects with normal cardiac systolic function may be due in part to renal impairment. With aging, biologic compensation of the cardiac natriuretic peptide system may be attenuated.

Neuropilin-2 is overexpressed in the rat brain after limbic seizures.

Structural rearrangement and synaptic reorganization are known to occur in the brain after seizures. If neuronal rearrangement after seizures always results in abnormal hyperexcitability, it would provide an accurate pathway to the appropriate target and as a result, it may be the mechanism of epileptogenesis. This study examined the mechanism of axon guidance in the mature rat brain after seizures by evaluating the expression of the axonal guidance molecule, neuropilin-2. We assessed the expression of neuropilin-2 by northern blotting and immunohistochemistry in rat with seizures created by kindling stimulation and kainate injection.The neuropilin-2 mRNA level was increased in the whole brain of the rats at 24 h after either type of seizure. Neuropilin-2 mRNA was not increased at 2 weeks after the last stimulation. Immunohistochemistry demonstrated that neuropilin-2 protein was increased in the dentate gyrus and the entorhinal cortex in the both seizure models. These findings suggested that there was overexpression of neuropilin-2 in the brains of mature rats with different types of seizure. Accordingly, neuropilin-2 might regulate remodeling after seizures as it does during the development of the hippocampal formation. Our findings suggest that axons may not project and outgrow 'aberrantly' after seizures, but may be regulated by the chemorepellent effect through neuropilin-2.