Historical perspective of neurology in Iran.

Iran is an ancient country, known as the cradle of civilization. The history of medicine in Iran goes back to the existence of a human in this country, divided into three periods: pre-Islamic, medieval, and modern period. There are records of different neurologic terms from the early period, while Zoroastrian (religious) prescription was mainly used until the foundation of the first medical center (Gondishapur). In the medieval period, with the conquest of Islam, prominent scientists were taught in Baghdad, like Avicenna, who referred to different neurologic diseases including stroke, paralysis, tremor, and meningitis. Several outstanding scientists developed the medical science of neurology in Iran, the work of whom has been used by other countries in the past and present. In the modern era, the Iranian Neurological Association was established with the efforts of Professor Jalal Barimani in 1991.

A case of Fragile X-associated tremor/ataxia syndrome (FXTAS).

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset neurodegenerative disorder associated with dysfunction of movement, memory, and the peripheral nervous system. We report an 82 years old male who presented with tremors and difficulty with balance that started at 65 years of age. His motor examination revealed decreased strength in left lower extremity. Tremors were seen in both the upper limbs at rest that worsened with movement. Bilateral lower extremities showed absent vibration and proprioception sensations, absent reflexes and upgoing toes. Electrodiagnostic studies revealed sensory predominant axonal sensory-motor peripheral polyneuropathy. Brain MRI revealed microvascular ischemic changes. The cervical and lumbar MRI showed diffuse degenerative changes. Genetic test for heritable causes of ataxia revealed a premutation in Fragile X gene (84 CGG repeats), confirming the diagnosis of FXTAS. On further genetic testing three out of his four daughters also tested positive for the FMR1 premutation. In appropriate clinical setting, Fragile X-associated tremor/ataxia syndrome (FXTAS) should be considered in every middle aged/elderly patient who presented with slowly progressive ataxia, tremor and peripheral polyneuropathy without any history of cognitive or neurological disabilities in childhood.

"Dystrophia Myotonica" and the Legacy of Hans Gustav Wilhelm Steinert.

The objective of this analysis is to study the life of Hans Gustav Wilhelm Steinert and his role in identifying several neurologic disorders including myotonic dystrophy (DM). DM type 1 (DM1) is a commonly inherited adult muscle disorder. In 1909, its characteristics were first described by Hans Steinert (1875-1911), a German neurologist. Born in Dresden, Germany, Steinert studied philosophy and medicine at the Universities of Leipzig, Berlin, Freiberg, and Kiel. There, under the supervision of Heinrich Curschmann, he accomplished his own works on aphthongia, cerebral muscular atrophy, and cerebral hemiplegia. In 1909, he published his study on six patients exhibiting characteristic myotonia. With autopsy findings of muscular fibrosis, Steinert identified this independent symptom complex as "Dystrophien Myotoniker" (DM). Overall, Steinert's accurate clinical characterization, coupled with the first ever autopsy finding of this condition, laid the foundations of our current understanding about DM1. This review serves as a tribute to the achievements of Hans Steinert and provides an opportunity to understand the historical perspective of DM1. Information for this analysis was gathered from PubMed and libraries at University of Southern California and University of California, Los Angeles. In addition, personal communications with Professor Benedikt Schoser at The University of Munich, Professor Tiemo Grimm at The University of Wuerzburg, and Professor Peter Harper at The University of Wales are acknowledged.

History of neuroimaging: the legacy of William Oldendorf.

The field of neuroimaging witnessed remarkable progress in the post-World War II era, resulting in tremendous benefits for healthcare today. One such important milestone was the development of the computerized axial tomography (CAT) scan. This state of the art technique has paved the way for modern diagnostic imaging like magnetic resonance imaging (MRI). Dr William Oldendorf, the first designer of axial tomography, is regarded by some as the true father of neuroimaging. However, because of various reasons, he was unable to succeed with his concept and was also unfortunately denied his due entitlement of the Nobel Prize for Medicine and Physiology in 1979. This article discusses his contributions to the fields of neuroimaging and neurosciences, along with the politics that surrounded the awarding of the Nobel Prize of 1979. Another associated topic that is touched on in this article is the ever-growing dispute over the interpretations of neuroimaging between neurologists and neuroradiologists.