Sturge Weber sendromunda erken infantil donemde nobetler goruldugunde, hemiparezinin erken donemd... more Sturge Weber sendromunda erken infantil donemde nobetler goruldugunde, hemiparezinin erken donemde gelistigi, nobetlerin direncli olabildigi ve mental motor retardasyonun ilerleyici oldugu bilinmektedir. Son zamanlarda bu olgula-ra erken cerrahi mudahale yapilmasi onerilmektedir. Bu yazida kompleks parsiyel nobetler seklinde direncli nobetler-le gelen ve basarili bir sekilde sol fonksiyonel hemisferektomi yapilan Sturge-Weber sendromlu bir olgu sunulmustur. 21 aylik kiz olgu 2.5 aylikken baslayan vucudun sag tarafina lokalize nobetler nedeniyle hastanemize getirildi. Gunde 10 defaya kadar olabilen bu nobetler atesli hastaliklari sirasinda statusa neden oluyordu. Fizik muayenesinde sol yuz yarisi ve boyunda hemajiomu mevcut olan hastanin sag hemiparezi ve mikrosefalisi mevcuttu. Beyin manyetik rezo-nans incelemesinde sol serebral hemisfer temporooksipital loblarda anjioma ve gliozis saptandi. Fenobarbital, karbamazepin ve valproat tedavileri maksimum dozlarda kullanilmasina ragmen 6 ...
Journal of Turgut Ozal Medical Center, Aug 1, 2008
Methyl malonic acidemia is a rare form of organic acidemias (that frequently observed with the ne... more Methyl malonic acidemia is a rare form of organic acidemias (that frequently observed with the neurological findings). It was noted that optic atrophy could be developed as a rare complication. An 8- year-old girl presented to our hospital with growth retardation, weight loss and vomiting. Her weight and length were (l3 P) and neutropenia was determined. During follow-up, metabolic acidosis was diagnosed in this patient after detection of acidotic respiration, (midriatic pupils, loss of light reflex and optic atrophy )- an indicative of optic nerve disease. Cranial MRI revealed mild atrophy and symmetrical hyperintensity in peritrigonal and lentiform nuclei in T2-weighted images. The patient had lactic acidemia and methyl malonic acidemia was detected with urinary organic acid screening. It was emphasized that methymalonic acidemia patients required to follow-up for optic nerve involvement. Key words: Methylmalonic Acidemia, Optic Nerve, Childhood.
Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating infection of the CNS that i... more Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating infection of the CNS that is characterized by multifocal white matter involvement often following an infection and vaccination. A 16-year-old child receiving risperidone for 7 years for the treatment of attention-deficit/hyperactivity disorder was hospitalized with complaints of double-vision during the past month and weakness on his right side, ataxia, and vomiting over the past 3 days. The child was diagnosed with ADEM after clinical, laboratory, and cranial MRI was conducted. Following an initial 3-day therapy with pulsed methylprednisolone, the child showed obvious clinical improvement. The treatment was continued with prednisolone and significant improvement was achieved. Enterovirus was detected in the results of the viral examination of the CSF. This child was found to be an interesting case having been diagnosed with ADEM associated with enteroviral infection, because of the rarity of few case reports in the literature.
Ketogenic diet (KD) remains a valuable treatment option for children with drug-resistant epilepsy... more Ketogenic diet (KD) remains a valuable treatment option for children with drug-resistant epilepsy. However, it may cause many well-known adverse effects such as dyslipidemia or kidney stones. But, its effects on thyroid functions are largely unknown. The aim of this study was to investigate the effects of the KD on thyroid functions in children with drug-resistant epilepsy. A total of 66 children (35 females) aged 3–193 months (median, 52 months) with drug-resistant epilepsy who received a KD for at least 12 months were enrolled in the study. All children were started on KD with 3:1 ratio which was then adjusted as clinically necessary. Serum free-thyroxine (FT4) and thyroid stimulating hormone (TSH) concentrations were measured before starting treatment and at the first, sixth and twelfth months of treatment. Changes in FT4 and TSH concentrations over 12 months were analyzed. Median serum FT4 and TSH concentrations, and the frequencies of patients with low FT4 and high TSH concentrations did not change significantly in the study sample over the 12-month study period. Serum FT4 levels increased significantly and TSH concentrations decreased insignificantly in four patients receiving L-thyroxine replacement therapy. During the 12-month treatment period, BMI-SDS increased, and the number of antiepileptic drugs decreased significantly. It appears that KD therapy does not impair thyroid functions in children with drug-resistant epilepsy. KD can be used safely along with L-thyroxine replacement even in children with pre-existing subclinical hypothyroidism.
21st International Congress of the World-Muscle-Society -- OCT 04-08, 2016 -- Granada, SPAINWOS: ... more 21st International Congress of the World-Muscle-Society -- OCT 04-08, 2016 -- Granada, SPAINWOS: 000384790100309World Muscle So
Sturge Weber sendromunda erken infantil donemde nobetler goruldugunde, hemiparezinin erken donemd... more Sturge Weber sendromunda erken infantil donemde nobetler goruldugunde, hemiparezinin erken donemde gelistigi, nobetlerin direncli olabildigi ve mental motor retardasyonun ilerleyici oldugu bilinmektedir. Son zamanlarda bu olgula-ra erken cerrahi mudahale yapilmasi onerilmektedir. Bu yazida kompleks parsiyel nobetler seklinde direncli nobetler-le gelen ve basarili bir sekilde sol fonksiyonel hemisferektomi yapilan Sturge-Weber sendromlu bir olgu sunulmustur. 21 aylik kiz olgu 2.5 aylikken baslayan vucudun sag tarafina lokalize nobetler nedeniyle hastanemize getirildi. Gunde 10 defaya kadar olabilen bu nobetler atesli hastaliklari sirasinda statusa neden oluyordu. Fizik muayenesinde sol yuz yarisi ve boyunda hemajiomu mevcut olan hastanin sag hemiparezi ve mikrosefalisi mevcuttu. Beyin manyetik rezo-nans incelemesinde sol serebral hemisfer temporooksipital loblarda anjioma ve gliozis saptandi. Fenobarbital, karbamazepin ve valproat tedavileri maksimum dozlarda kullanilmasina ragmen 6 ...
Journal of Turgut Ozal Medical Center, Aug 1, 2008
Methyl malonic acidemia is a rare form of organic acidemias (that frequently observed with the ne... more Methyl malonic acidemia is a rare form of organic acidemias (that frequently observed with the neurological findings). It was noted that optic atrophy could be developed as a rare complication. An 8- year-old girl presented to our hospital with growth retardation, weight loss and vomiting. Her weight and length were (l3 P) and neutropenia was determined. During follow-up, metabolic acidosis was diagnosed in this patient after detection of acidotic respiration, (midriatic pupils, loss of light reflex and optic atrophy )- an indicative of optic nerve disease. Cranial MRI revealed mild atrophy and symmetrical hyperintensity in peritrigonal and lentiform nuclei in T2-weighted images. The patient had lactic acidemia and methyl malonic acidemia was detected with urinary organic acid screening. It was emphasized that methymalonic acidemia patients required to follow-up for optic nerve involvement. Key words: Methylmalonic Acidemia, Optic Nerve, Childhood.
Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating infection of the CNS that i... more Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating infection of the CNS that is characterized by multifocal white matter involvement often following an infection and vaccination. A 16-year-old child receiving risperidone for 7 years for the treatment of attention-deficit/hyperactivity disorder was hospitalized with complaints of double-vision during the past month and weakness on his right side, ataxia, and vomiting over the past 3 days. The child was diagnosed with ADEM after clinical, laboratory, and cranial MRI was conducted. Following an initial 3-day therapy with pulsed methylprednisolone, the child showed obvious clinical improvement. The treatment was continued with prednisolone and significant improvement was achieved. Enterovirus was detected in the results of the viral examination of the CSF. This child was found to be an interesting case having been diagnosed with ADEM associated with enteroviral infection, because of the rarity of few case reports in the literature.
Ketogenic diet (KD) remains a valuable treatment option for children with drug-resistant epilepsy... more Ketogenic diet (KD) remains a valuable treatment option for children with drug-resistant epilepsy. However, it may cause many well-known adverse effects such as dyslipidemia or kidney stones. But, its effects on thyroid functions are largely unknown. The aim of this study was to investigate the effects of the KD on thyroid functions in children with drug-resistant epilepsy. A total of 66 children (35 females) aged 3–193 months (median, 52 months) with drug-resistant epilepsy who received a KD for at least 12 months were enrolled in the study. All children were started on KD with 3:1 ratio which was then adjusted as clinically necessary. Serum free-thyroxine (FT4) and thyroid stimulating hormone (TSH) concentrations were measured before starting treatment and at the first, sixth and twelfth months of treatment. Changes in FT4 and TSH concentrations over 12 months were analyzed. Median serum FT4 and TSH concentrations, and the frequencies of patients with low FT4 and high TSH concentrations did not change significantly in the study sample over the 12-month study period. Serum FT4 levels increased significantly and TSH concentrations decreased insignificantly in four patients receiving L-thyroxine replacement therapy. During the 12-month treatment period, BMI-SDS increased, and the number of antiepileptic drugs decreased significantly. It appears that KD therapy does not impair thyroid functions in children with drug-resistant epilepsy. KD can be used safely along with L-thyroxine replacement even in children with pre-existing subclinical hypothyroidism.
21st International Congress of the World-Muscle-Society -- OCT 04-08, 2016 -- Granada, SPAINWOS: ... more 21st International Congress of the World-Muscle-Society -- OCT 04-08, 2016 -- Granada, SPAINWOS: 000384790100309World Muscle So
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