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Zaki, M. S., A. Abdel-Aleem, G. Abdel-Salam, S. E. Marsh, J. L. Silhavy, A. J. Barkovich, M. E. Ross, S. N. Saleem, W. B. Dobyns, and J. G. Gleeson, "The molar tooth sign A new Joubert syndrome and related cerebellar disorders classification system tested in Egyptian families", Neurology, vol. 70, no. 7: Lippincott Williams & Wilkins, pp. 556–565, 2008. Abstract

Joubert syndrome and related cerebellar disorders (JSRD) are a group of recessive congenital ataxia conditions usually showing neonatal hypotonia, dysregulated breathing rhythms, oculomotor apraxia, and mental retardation. The pathognomonic finding in JSRD is the unique molar tooth sign (MTS) on brain imaging. There is a tremendously broad spectrum of signs and symptoms mainly including kidney, retina, and liver disease, along with polydactyly and facial dysmorphisms. Here we propose a new diagnostic classification within JSRD that includes four major subtypes. To test this classification, we performed a systematic recruitment and genetic evaluation from a single referral center in Egypt. Thirteen families were identified, four showed evidence of linkage to one of the four known genetic loci, three showed novel AHI1 mutations, and nine were excluded from known loci. Each family could be classified into one of the four subtypes. This classification may thus be useful in the evaluation of patients with JSRD.

GLOSSARY: BUN = blood urinary nitrogen; COACH = cerebellar vermis hypo/aplasia-oligophrenia-ataxia-ocular coloboma-hepatic fibrosis; CORS = cerebello-oculo-renal syndrome; CVH = cerebellar vermis hypoplasia; DAS = Dekaban-Arima syndrome; DWM = Dandy-Walker malformation; JSRD = Joubert syndrome and related cerebellar disorders; LCA = Leber congenital amaurosis; lod = logarithm of odds score; MKS = Meckel-Gruber syndrome; MTS = molar tooth sign; NPH = nephronophthisis; NRC = National Research Center; OFD-VI = oro-facio-digital syndrome type VI; PCH = pontocerebellar hypoplasia; RHO = rhombencephalosynapsis; SLS = Senior-Loken syndrome.

Zaki, M. S., S. N. Saleem, W. B. Dobyns, A. J. Barkovich, H. Bartsch, A. M. Dale, M. Ashtari, N. Akizu, J. G. Gleeson, and A. M. Grijalvo-Perez, "Diencephalic–mesencephalic junction dysplasia: a novel recessive brain malformation", Brain, vol. 135, no. 8: Oxford University Press, pp. 2416–2427, 2012. Abstract

We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic–mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic–mesencephalic junction with a characteristic ‘butterfly’-like contour of the midbrain on axial sections. Additional imaging features included variable degrees of supratentorial ventricular dilatation and hypoplasia to complete agenesis of the corpus callosum. Diffusion tensor imaging showed diffuse hypomyelination and lack of an identifiable corticospinal tract. All patients displayed severe cognitive impairment, post-natal progressive microcephaly, axial hypotonia, spastic quadriparesis and seizures. Autistic features were noted in older cases. Talipes equinovarus, non-obstructive cardiomyopathy and persistent hyperplastic primary vitreous were additional findings in two families. One of the patients required shunting for hydrocephalus; however, this yielded no change in ventricular size suggestive of dysplasia rather than obstruction. We propose the term ‘diencephalic–mesencephalic junction dysplasia’ to characterize this autosomal recessive malformation.

Zaki, M. S., G. M. H. Salam, S. N. Saleem, W. B. Dobyns, M. Y. Issa, S. Sattar, and J. G. Gleeson, "New recessive syndrome of microcephaly, cerebellar hypoplasia, and congenital heart conduction defect", American Journal of Medical Genetics Part A: Wiley Online Library, 2011. Abstract
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Youssef, A., S. Zagonari, G. Salsi, S. N. Saleem, J. Krsmanovic, and G. Pacella, "Prenatal diagnosis of isolated butterfly vertebra", Ultrasound in Obstetrics & Gynecology, vol. 44, issue 6, pp. 26-27, 2014.
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Wade, A. D., R. Beckett, G. Conlogue, G. Garvin, S. Saleem, G. Natale, D. Caramella, and A. Nelson, "Diagnosis by consensus: A case study in the importance of interdisciplinary interpretation of mummified remains.", International journal of paleopathology, vol. 24, issue March, pp. 144-153, 2018 Oct 30, 2019. Abstract

OBJECTIVE: The goal of this study is to demonstrate the need for interdisciplinary consensus and inclusion of mummy radiology specialists in analyses of mummified remains.

MATERIALS: This study uses paleoimaging data for an ancient Egyptian mummy at the Museum of Human Anatomy "Filippo Civinini".

METHODS: This study demonstrates the benefit of evaluation of mummified remains in a multi-disciplinary interpretive team.

RESULTS: The authors propose a diagnosis of DISH, additional signs of undifferentiated spondyloarthropathy, and lumbarisation of S1.

CONCLUSIONS: The process of diagnosis by consensus is essential to the analysis of mummified remains, which are complexly altered through natural and anthropogenic processes in the millennia subsequent to the individual's death.

SIGNIFICANCE: Mummy paleoimaging and paleopathology lacks a unifying set of standards. We present an example of the value to be found in the multi-disciplinary diagnosis by consensus approach.

LIMITATIONS: We discuss numerous challenges to accurate and meaningful interpretation that radiography of mummified remains pose.

SUGGESTIONS FOR FURTHER RESEARCH: While the authors do not seek to impose any single set of standards, we do recommend a larger discussion on the topic of (culture-specific) standardisation in mummy paleoimaging and paleopathology. We further recommend the development of an international, multi-disciplinary panel of paleoimaging interpreters.

Wade, A. D., R. Beckett, G. Conlogue, G. Garvin, S. Saleem, G. Natale, D. Caramella, and A. Nelson, "Diagnosis by consensus: Interpreting mummified pathological conditions", American Journal of Physical Anthropology, vol. 156, pp. 315-316, 2015.
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SN, S., S. YY, and S. AS, ". Radiology Education in the Faculty of Medicine at Cairo University (Kasr Al-Ainy Hospital). ", Radiology Education: The Scholarship of Teaching and learning, Berlin Heidelberg , Springer-Verlag Berlin Heidelberg , 2008.
SN, S., "Fetal Cardiac Magnetic Resonance (CMR)", Echocardiography - New Techniques: InTech, 2012. CU-PDF
SN, S., and S. YY, "Measuring competence of Radiology Education Programs and Residents: The Egyptian Experience", Radiology Education: The Evaluation and Assessment of Clinical Competence. , Berlin Heidelberg , Springer-Verlag Berlin Heidelberg , 2012. AbstractCU-PDF

Ancient Egypt had an advanced elaborate medical education and practice ruled by a competent bureaucracy that apprenticed physicians to be practicing healers. In modern history, the Faculty of Medicine at Cairo University (Kasr Al-Ainy), established in 1827, continues the glory of Egypt in medical education as one of the biggest and oldest medical schools in Africa and the Middle East. Its central Radiology Department, with its total 77 radiologists, is responsible for clinical services as well as for providing multiple calibre radiology education programs for about 100 trainees annually from Egypt and neighbouring countries. Radiology education programs are planned for radiology residents to obtain master’s degree (M.Sc.), for assistant lecturers to obtain medical doctorate (M.D.) and for visitor trainees. Objectives of radiology education programs include knowledge, practical skills, intellectual capabilities and communications with medical societies and communities. Trainees are assessed to determine if learning objectives have been fulfilled on a daily, weekly and biannual basis. Radiology education programs are measured for professional performance through the university’s self-assessment studies; national assessment is measured through the National Authority for Quality Assurance and Accreditation in Education (NAQAAE), Egypt, and international assessment is measured through the World Federation for Medical Education (WFME).

Shokry, M., and S. Saleem, "IC Conferences", Middle East Fertility Society Journal, vol. 5, no. 3, pp. 231–235, 2000. Abstract
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Shokry, M., and S. Saleem, "IC Conferences", Middle East Fertility Society Journal, vol. 5, no. 3, pp. 231–235, 2000. Abstract
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Selim, L. A., M. S. Zaki, H. A. Hussein, S. N. Saleem, A. S. Kotoury, and M. Y. Issa, "Developmental abnormalities of mid and hindbrain: A study of 23 Egyptian patients", Egyptian Journal of Medical Human Genetics, vol. 9, no. 2, pp. 215–236, 2009. Abstract
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Selim, L. A., M. S. Zaki, H. A. Hussein, S. N. Saleem, A. S. Kotoury, and M. Y. Issa, Developmental abnormalities of mid and hindbrain: Astudy of 23, , 2008. Abstract

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Saleem, S. N., "Feasibility of MRI of the fetal heart with balanced steady-state free precession sequence along fetal body and cardiac planes", American Journal of Roentgenology, vol. 191, no. 4: Am Roentgen Ray Soc, pp. 1208–1215, 2008. Abstract
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Saleem, S. N., and Z. Hawass, "Variability in Brain Treatment During Mummification of Royal Egyptians Dated to the 18th–20th Dynasties: MDCT Findings Correlated With the Archaeologic Literature", American Journal of Roentgenology, vol. 200, issue 4, pp. W336-W344, 2013. Abstract

OBJECTIVE. The objective of our study was to use MDCT to study brain treatment and removal (excerebration) as part of mummification of royal Egyptian mummies dated to the 18th to early 20th Dynasties and to correlate the imaging findings with the archaeologic literature.

MATERIALS AND METHODS. As part of an MDCT study of the Royal Ancient Egyptian Mummies Project, we analyzed CT images of the heads of 12 mummies dated to circa 1493–1156 BC (18th to early 20th Dynasties). We reconstructed and analyzed CT images for the presence of cranial defects, brain remnants, intracranial embalming materials, and nasal packs. We compared the CT findings of mummies dated to the 18th Dynasty with those dated to the 19th to early 20th Dynasties.

RESULTS. The Akhenaten mummy was excluded because of extensive postmortem skull fractures. CT showed that no brain treatment was offered to three mummies (Thutmose I, II, and III) who dated to the early 18th Dynasty and was offered to the eight mummies who dated later. The route of excerebration was transnasal in eight mummies; an additional suspected route was via a parietal defect. CT showed variable appearances of the intracranial contents. There were larger volumes of cranial packs and more variability in the appearances of the cranial packs in the royal mummies dated to the 19th to 20th Dynasties than in those dated to the 18th Dynasty.

CONCLUSION. MDCT shows variations in brain treatment during mummification of royal Egyptian mummies (18th–20th Dynasties). This study sets a template for future CT studies of the heads of ancient Egyptian mummies and focuses on the key elements of cranial mummification in this ancient era.

Saleem, S. N., and M. S. Zaki, "Role of MR imaging in prenatal diagnosis of pregnancies at risk for Joubert syndrome and related cerebellar disorders", American Journal of Neuroradiology, vol. 31, no. 3: Am Soc Neuroradiology, pp. 424–429, 2010. Abstract
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Saleem, S. N., "MR Imaging Diagnosis of Uterovaginal Anomalies: Current State of the Art1", Radiographics, vol. 23, no. 5: Radiological Society of North America, pp. e13–e13, 2003. Abstract
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Saleem, S. N., and Z. Hawass, "Computed tomography study of the feet of mummy of Ramesses III: New insights on the Harem Conspiracy", J Comput Assist Tomogr , vol. Sept , issue 41, pp. 15-17, 2017.
Saleem, S. N., "Feasibility of MRI of the fetal heart with balanced steady-state free precession sequence along fetal body and cardiac planes", American Journal of Roentgenology, vol. 191, no. 4: Am Roentgen Ray Soc, pp. 1208–1215, 2008. Abstract
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Saleem, S. N., Y. Y. Sabri, and A. S. Saeed, "Radiology Education in the Faculty of Medicine at Cairo University", Radiology Education: The Scholarship of Teaching and Learning: Springer, pp. 283, 2008. Abstract
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Saleem, S. N., "MR Imaging Diagnosis of Uterovaginal Anomalies: Current State of the Art1", Radiographics, vol. 23, no. 5: Radiological Society of North America, pp. e13–e13, 2003. Abstract
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Saleem, S. N., and Z. Hawass, "Subcutaneous packing in Royal Egyptian mummies dated from 18th to 20th Dynasties", J Comput Assist Tomogr , vol. 39, issue 3, pp. 301-306, 2015.
Saleem, S. N., and Y. Y. Sabri, "Measuring Competence of Radiology Education Programs and Residents: The Egyptian Experience", Radiology Education: Springer Berlin Heidelberg, pp. 129–141, 2012. Abstract
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Saleem, S. N., A. H. Said, M. Abdel-Raouf, E. A. El-Kattan, M. S. Zaki, N. Madkour, and M. Shokry, "Fetal MRI in the evaluation of fetuses referred for sonographically suspected neural tube defects (NTDs): impact on diagnosis and management decision", Neuroradiology, vol. 51, no. 11: Springer, pp. 761–772, 2009. Abstract
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Saleem, S. N., and Z. Hawass, "Ankylosing spondylitis or diffuse idiopathic skeletal hyperostosis (DISH) in Royal Egyptian mummies of 18th-20th Dynasties? CT and archaeology studies", Arthritis and Rheumatology , vol. 66, issue 12, pp. 3311-3316, 2014.
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