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Biomedical subjects

Yuki Anzai

Publications and source records attributed to Yuki Anzai.

5 recordsLinked to original sources

Zonisamide eradicated paroxysmal headache with EEG abnormalities triggered by hypertensive encephalopathy due to purpura nephritic syndrome.

Generally, prognosis of hypertensive encephalopathy in childhood is favorable. We reported a 5-year-old girl who presented with a headache attack and EEG abnormalities after hypertensive encephalopathy due to purpura nephritis. The patient had suffered from hypertensive encephalopathy due to purpura nephritis, which soon ameliorated. Five months later, she developed attacks of headache, vomiting and disturbed consciousness with left side-predominant EEG abnormalities. Although carbamazepine and sodium valproate failed to improve her condition, zonisamide eradicated both the symptoms and EEG abnormalities, and an attack has not reoccurred for 5 years since completion of her treatment. It is noteworthy that delayed-onset complications can occur in child hypertensive encephalopathy, cases of which should be followed up prudently. Zonisamide should be considered for treatment of attacks of headaches with an epileptic character.

Anticonvulsants↗

Protracted juvenile neuronal ceroid lipofuscinosis--an autopsy report and immunohistochemical analysis.

The juvenile form of neuronal ceroid lipofuscinosis (JNCL) is caused by mutations in the CLN3 gene, and is characterized by progressive loss of vision and development of motor deficits. A few patients exhibit a more protracted clinical course and are diagnosed with protracted JNCL (PJNCL). Here, we report the autopsy in a case of PJNCL in a 55-year-old male and immunohistochemical examination of the involvement of oxidative stress and glutamate excitotoxicity in neurodegeneration. The patient was born to consanguineous parents (I assume this means that the parents were related. If not, then the sentence will need to be changed again.) and had brothers with similar neurological disease. He showed mental retardation and visual impairment in the first decade which gradually developed along with motor dysfunction for over 40 years. At autopsy, the cerebral pyramidal neurons revealed deposition of lipopigments, which demonstrated 'finger print' and curvilinear profiles on electron microscopy. He also exhibited cerebellar cortical atrophy, fibrillary gliosis in the white matter, and rarefication in the globus pallidus. Immunohistochemically, the number of neurons immunoreactive for advanced glycation end product was elevated in the cerebellar cortex and midbrain. Immunoreactivity for excitatory amino acid transporter 1 was reduced in the cerebellar dentate and inferior olivary nuclei. These findings suggest that oxidative damage to proteins and disturbed glutamate transport can be involved in PJNCL.

8-Hydroxy-2'-Deoxyguanosine↗

[Treatment of sleep apnea syndrome in a Down syndrome patient with behavioral problems by noninvasive positive pressure ventilation: a successful case report].

The use of noninvasive positive pressure ventilation (NPPV) is increasingly accepted as a treatment of respiratory problems in patients with neuromuscular disease. However, its use in mentally retarded and un-cooperative patients has not been reported. We report here the evaluation and treatment of sleep apnea in a Down syndrome patient. After recovering from a life threatening respiratory failure the patient had persistent sleep apnea syndrome. Limited examinations disclosed that he had two types of apnea; obstructive and central type apnea. Our treatment was a diet with intake restricted up to 1,000 kcal per day for the obstructive apnea, and NPPV with low dosage of oxygen for the obstructive and central apnea. With these treatments in one year's hospitalization, his quality of life was significantly improved. He has continued the treatment in the outpatient department and enjoys a better quality of life both at home and in the community. The successful treatment of this case may become an example of the more extensive use of NPPV for such respiratory problems in handicapped children and adults with behavioral problems.

Adult↗

Fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability: coexistence of auditory neuropathy and cochlear hearing loss.

We encountered a 10-year-old girl with fluctuating sensorineural hearing loss, episodic headache, and white matter stroke. Strenuous exercise, febrile illness, and general anesthesia all temporarily worsened hearing. Audiologic findings were asymmetric: left-sided retrocochlear dysfunction consistent with auditory neuropathy contrasted with cochlear hearing loss in the right ear. Platelets obtained during a headache-free period showed excessive responsiveness to collagen in vitro, while episodic elevations of thromboxane B(2) and thrombin-antithrombin III complex were noted in blood sampled during headache. Treatment of hyperaggregability of platelets with aspirin and antioxidant vitamins relieved headache, while adenosine triphosphate administration improved hearing thresholds. In this patient, hearing impairment and white matter strokes appeared to respectively related to impaired blood flow to the cochlea and white matter caused by platelet dysfunction triggered by physiologic stresses.

Adenosine Triphosphate↗

[An infant with diffuse axonal injury].

Development of MRI enabled accurate and rapid diagnosis of head traumas which had been impossible with the conventional CT. The diagnosis of posttraumatic diffuse axonal injury (DAI) is a typical example. Most of the reported cases of DAI in childhood are of relatively older age, and DAI is rare in infancy. We report here a 1-year-9-month-old infant with DAI. After falling from two meters in height, he presented consciousness disturbance and a seizure that necessitated artificial respiration for half a day. He recovered completely without sequelae. Brain CT on arrival to the ER department was unremarkable, but MRI scans shortly thereafter showed typical findings of DAI. In childhood, DAI tends to be milder than in adult-hood, which may be explained by the age-dependent frequency of traffic accidents, and by anatomical and functional factors of a infant's brain. A large number of infantile cases should be accumulated to draw a more clearcut conclusion.

Brain↗