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Farideh Nejat and Behzad Eftekhar

✓ This 9-year-old girl with rapidly progressive cerebral demyelinating disease presented with hemiplegia and intracranial hypertension. Brain images revealed four lesions with mass effect in the subcortical white matter of both hemispheres. Demyelination was found on pathological studies of these lesions. The patient experienced some recovery with corticosteroid treatment but improved completely with decompressive aspiration of the largest lesion.

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Farideh Nejat and Syed Shuja Kazmi

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Farid Radmanesh, Farideh Nejat and Maryam Monajemzadeh

✓Encephalocele is a cystic congenital malformation in which central nervous system structures herniate through a defect in the cranium. The coincidence of encephalocele and neoplasm is a very rare condition. The authors present the case of a 5-month-old girl who underwent surgery for encephalocele and whose postoperative histopathological assessment revealed evidence of a mature teratoma inside the lesion. The embryogenesis of such a lesion is discussed.

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Farideh Nejat and Mostafa El Khashab

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Saeed Ansari, Majid Dadmehr and Farideh Nejat

✓ Occipital dermal sinuses (ODSs) are congenital lesions located in the midline and characterized by a cutaneous pit or dimple. The intracranial extension as well as the associated symptoms are variable. To date, a familial occurrence of these lesions has not been reported. In this paper the authors report on a 2-year-old boy with an ODS and intracranial hypertension. The boy’s mother had a similar lesion but did not have any complaints. Following their experience with this case and a literature review, the authors suggest that there may be a genetic basis in certain instances of ODS.

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Thomas Pittman

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James T. Goodrich

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Saeed Ansari, Farideh Nejat, Shahrooz Yazdani and Majid Dadmehr

Object

The coexistence of split cord malformations (SCMs) and myelomeningoceles (MMCs) can be misdiagnosed or ignored and can cause spinal cord tethering. The authors investigated cases involving the coexistence of SCMs and MMCs in patients from the Children's Hospital Medical Center in Tehran.

Methods

Of the 330 patients with MMCs who underwent operations at the Children's Hospital Medical Center between January 2001 and June 2005, 33 (10%) had an associated SCM. These 33 cases were retrospectively reviewed.

Results

Eighteen patients (55%) were female, and the mean age of the patients at presentation was 2.9 months. In 17 of the 33 patients, the SCM occurred at the level of the neural placode. A Type I SCM was found in 26 children. Two patients had hypertrichosis. Eight patients had unilateral leg paresis. The MMC sac was located in the lumbar region in 14 cases. Two patients had double spinal dysraphism with meningoceles at the thoracic level. All patients underwent simultaneous repair of both lesions at the time of surgery.

Conclusions

Patients with MMCs, especially those with unusual manifestations such as unilateral paresis or skin lesions, should undergo a preoperative clinical examination to check for the presence of an SCM. Use of spinal magnetic resonance imaging can help in identifying the associated abnormalities. The neural placode and the rostral and caudal spinal cord segments should be carefully inspected for dysraphic lesions such as SCMs during the operation to repair the MMC. In this paper, the authors hope to show the wisdom of identifying these anomalies at the time of the initial repair of the MMC.

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Farideh Nejat, Samira Zabihyan Cigarchi and Syed Shuja Kazmi

✓The authors describe the case of a 2-year-old boy who experienced progressive spastic paraparesis for several months. Magnetic resonance imaging revealed an extensive extradural arachnoid cyst at the T3–L1 levels and posterior spinal cord herniation at T3–4. Surgical release of the neck of the hernia and total resection of the arachnoid cyst were performed. The patient had good clinical recovery several weeks after surgical decompression. This case highlights an exceedingly rare type of spinal cord herniation in a pediatric age group, and focuses on the abnormalities of the dorsal dura mater, together with imaging and intraoperative findings.