Cleidocranial Dysplasia: Presentation of Clinical and Radiological Features of a Rare Syndromic Entity.

Cleidocranial dysplasia is a developmental anomaly of the skeleton and the teeth. This condition may be inherited and be transmitted as dominant characteristics in either gender, or may appear spontaneously. It presents with skeletal defects of several bones, such as partial or complete absence of clavicles, late closure of the fontanels, presence of open skull sutures and multiple wormian bones. The dental manifestations are mainly delayed exfoliation of primary teeth and delayed eruption of permanent teeth with multiple impacted supernumerary teeth. This case of a 20 year old girl is noteworthy to the dentist as it deals with clinical and radiological features (a high number of impacted and supernumerary teeth as well as brachycephaly, frontal bossing and hypermobility of shoulders) which may come handy in clinical practice.

Peripheral and Central Nervous System Involvement in Recently Diagnosed Cases of Hypothyroidism: An Electrophysiological Study.

BACKGROUND: Hypothyroidism, one of the most common endocrine disorders, may induce neurological abnormalities at an early stage of the disease. AIM: The study was designed to assess the electrophysiological alterations of some selected variables of nerve conduction, brainstem auditory evoked potentials (BAEPs), and visual evoked potentials (VEPs) in hypothyroid patients. SUBJECTS AND METHODS: Sixty patients of newly diagnosed hypothyroidism and an equal number of age-matched controls were selected for the study. Nerve conduction studies that included parameters as latencies, conduction velocities, and amplitude of motor nerves, i.e., median, ulnar, common peroneal, tibial nerve, and sensory nerves, i.e., median and sural nerves was performed in both hypothyroid patients and controls. Further, BAEPs and VEPs of all the patients were done. The data were compiled and statistically analyzed using Student's unpaired t-test to observe any electrophysiological alterations in hypothyroid patients as compared to healthy controls. RESULTS: On comparative evaluation, statistically significant increase in latency of median, ulnar, tibial, and sural nerves; decrease in conduction velocities of all the tested nerves and decrease in amplitude of median, tibial, and sural nerves was observed in hypothyroid patients. Statistically significant increase in latencies, interpeak latencies, and decrease in amplitudes of BAEP waves and statistically significant increase in P100 latency of VEP was seen in hypothyroid patients. CONCLUSION: The results of our study suggest that peripheral and central neuropathy develops in patients of hypothyroidism at an early stage of disease and the electrophysiological investigations of such patients can help in timely detection and treatment of neurological disorders that occur due to thyroid hormone deficiency.