Fatal insomnia: the elusive prion disease

A previously well 54- year-old woman presented with a short history of diplopia, cognitive decline, hallucinations and hypersomnolence. The patient had progressive deterioration in short-term memory, ocular convergence spasm, tremor, myoclonus, gait apraxia, central fever, dream enactment and seizures. Results of investigations were normal including MRI brain, electroencephalogram, cerebrospinal fluid (CSF, including CSF prion protein markers) and brain biopsy. The patient died from pneumonia and pulmonary embolus. Brain postmortem analysis revealed neuropathological changes in keeping with Fatal familial insomnia (FFI); the diagnosis was confirmed on genetic testing. FFI is caused by an autosomal dominant and highly penetrant pathogenic Prion Protein gene PRNP. Although usually familial, fatal insomnia (FI) also occurs in a rare sporadic form. FI is a rare human prion disease with prominent sleep disturbance, autonomic, motor, cognitive and behavioural involvement. Patient management is with best supportive care and early suspected diagnosis allows for timely palliation.

Dental Occlusion and Ophthalmology: A Literature Review

Stomatognathic system is strictly correlated to other anatomical regions; many studies investigated relationship between temporomandibular joint and posture, several articles describe cranio-facial pain from dental causes, such as trigger points. Until now less interest has been given to connections between dental occlusion and ophthalmology, even if they are important and involving. Clinical experience in dental practice claims that mandibular latero-deviation is connected both to eye dominance and to defects of ocular convergence. The trigeminal nerve is the largest and most complex of the twelve cranial nerves. The trigeminal system represents the connection between somitic structures and those derived from the branchial arches, collecting the proprioception from both somitic structures and oculomotor muscles. The intermedius nucleus of the medulla is a small perihypoglossal brainstem nucleus, which acts to integrate information from the head and neck and relays it on to the nucleus of the solitary tract where autonomic responses are generated. This intriguing neurophysiological web led our research group to investigate anatomical and functional associations between dental occlusion and vision. In conclusion, nervous system and functional pathways strictly connect vision and dental occlusion, and in the future both dentists and oculists should be more and more aware of this correlation for a better diagnosis and therapy.