Terms such as retrosternal, substernal, intrathoracic, or mediastinal have been used to describe a goitre that extends beyond the thoracic inlet. However, there is a lack of consensus regarding the exact definition of a retrosternal goitre (RSG).(1) The majority of patients present with shortness of breath or asthma like symptoms (68.8%), as was the case in the studied patient. Other modes of presentation include neck mass (75%), hoarseness of voice (37.5%), dysphagia (31.3%), stridor/wheezing (19%), or SVC obstruction. Upper airway obstruction due to thyroid gland has been reported up to 31%(2) and difficulty in intubation has been reported in 11%.(3) Central airway obstruction produces symptoms of dyspnoea, stridor, or obstructive pneumonia and is often misdiagnosed as asthma.(4) The CT scan was the most useful tool showing the nature and extent of the lesion in the reported case. In a recent publication, the CT scan was considered the gold-standard preoperative radiological investigation.(5) Surgery is the only effective treatment for retrosternal goitres. In most cases, suppressive therapy with thyroxine is ineffective in reducing the size of multinodular goitres;(7,8) radio-iodine therapy is both generally ineffective in large goitres(8) and may induce acute inflammation and swelling of the gland with the potential for airway obstruction. The operation of choice is usually a total thyroidectomy. Only around 2% of patients undergoing thyroidectomy for retrosternal goitre will require surgical access other than a standard collar incision (either manubriotomy, sternotomy or thoracotomy).(9) CONCLUSION: Despite all the advances in investigative modalities, retrosternal goitre still exists in 20% of patients over 70 years in endemic regions. It has to be recognised that it can be a cause of obstructive sleep apnoea. Early detection and prompt management goes a long way in decreasing the morbidity and mortality in patients with RSG.
Creutzfeld -Jacob Disease (CJD) is a rare type of neurodegenerative disease caused due to prion disease and is rapidly progressive and fatal. It is usually suspected based on the typical presenting symptoms, most important symptom being rapidly progressive dementia. Cases with atypical presentation are usually missed due to low clinical suspicion.
We present a case report of a 64-year-old male who presented with worsening cerebellar ataxia and only mild dementia.MRI done to rule out any organic cause, revealed classic findings of CJD.Following this EEG was performed. MRI helped in clinching the diagnosis in this case of low clinical suspicion. This was an extremely rare variant of CJD-the brownell openheimer variant.(1)
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.