This chapter brings together literary responses to the ruins of Rome. Over the centuries after Petrarch, the ruins had acquired historical, cultural and aesthetic validation, all the outcome of the development of a sentiment favourable to ruination; in short, ruin-mindedness. For an emotional validation we must turn to writers, who put into plain words how they felt about the ruins. The feelings are surprisingly various: sometimes elation, sometimes moral disgust. Whatever the reaction, it is usually founded, as was Petrarch’s, on the fact that the ruins of Rome have a historical and cultural context, thanks to the survival of Latin literature. The physical remains of the ancient city are given meaning by the Roman literary heritage, and it is that above all which enables writers to record a varied range of nuanced responses to them that are not likely to be evoked by a ruin without a history. Reactions to the ruins are affected by shifts in sensibility, especially the influence of romanticism, which insisted upon recording impressions of the ruins in moonlight. The ruins of Rome are signs to be interpreted in endless ways. This cannot be said of any other ruins anywhere.

In this chapter, we review the neuro-ophthalmologic history and examination, and provide frameworks for their assessment. We summarize the neuro-ophthalmologic manifestations in common movement disorders. Attention to the neuro-ophthalmologic system can assist with the differential diagnosis of these conditions.

Movement disorders arise from dysfunctional physiology within the motor and movement systems of the nervous system, and can involve multiple anatomic locations. A myriad of electrophysiologic manifestations can be detected in electromyography (EMG), electroencephalography (EEG), and other methods. Technical factors must be carefully considered and technical quality should be monitored throughout. Surface EMG provides the basis for the electrophysiologic examination of movement disorders. EEG is important for establishing cortical genesis as well as consciousness state determination during the movement disorder. Tremors of different etiologies may have different frequencies and activation characteristics that are best discovered on analysis of surface EMG characteristics. Also, classification of myoclonus physiology needs electrophysiologic testing. Proper myoclonus classification forms the best approach to symptomatic treatment strategy. Results from this testing provide important supplemental information, which can be used for a more exact diagnosis that leads to treatment.

Clinical evaluation of motor dysfunction is crucial to make a correct diagnosis. The gold standard is clinical evaluation by a movement disorder specialist, relying on subjective measures and patient report. Regular clinical assessments are needed to provide long-term measures that monitor motor progression over time and therapy response, not only in clinical settings but also during daily activities at home. Wearable sensors have been developed to assess objective and quantifiable measures of motor dysfunction. Such sensors are small, light, cheap and portable, containing built-in accelerometers and gyroscopes and data storage. These new technologies are revolutionizing the field of movement disorders to improve clinical diagnosis and evaluation, treatment monitoring at home, and progression of symptoms over time. They are also of interest for adaptive therapy options, e.g. closed-loop deep brain stimulation, and are successful in quantifying and measuring tremor, showing promise in assessing bradykinesia, dyskinesia, gait impairments and prediction of therapy response. Despite device development, there is no validated clinical application yet; further research is needed.

This chapter summarizes the functional–anatomic organization of the connectivity of the basal ganglia with the thalamocortical systems and the brainstem. This connectional organization substantiates the neural basis for the wide array of functions in which the basal ganglia are involved, ranging from pure sensorimotor to cognitive–executive and emotional–motivational behaviors. Across this broad array of motor and behavioral functions, the mechanism by which the basal ganglia contribute to these functions is through “response selection.” This mechanism fits well with the arrangement of the intrinsic connections between the individual basal ganglia nuclei, supporting the selection of appropriate responses in a particular context and, at the same time, the suppression of inadequate responses. A variety of symptoms as part of neurologic movement disorders, such as Parkinson’s disease, Huntington’s disease and dystonia, or neuropsychiatric diseases like obsessive-compulsive disorder, mood disorders, and drug addiction, might be interpreted as an inadequate selection of motor, cognitive, or affective responses to internal or external stimuli.

Tics are brief, sudden, non-rhythmic, repetitive movements. Tics can be motor or vocal. Further, both motor and vocal tics can be either simple or complex. Simple tics typically involve only one group of muscles and are brief and meaningless, whereas complex tics may last longer and appear more purposeful. Tic disorders usually begin in childhood and are classified according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) into four groups: (1) provisional tic disorder, (2) chronic motor or vocal tic disorder, (3) Tourette disorder (or Gilles de la Tourette syndrome), (4) tic disorder not otherwise specified. As tics can resemble almost any other movement disorder, phenotypic analysis alone is insufficient and patients must be questioned whether the execution is preceded by a premonitory sensation (urge to do, urge to move) and whether a temporary control of the movement can be achieved. Also, relief following execution of the tic is frequently reported. There are no biomarkers available for tics and diagnosis therefore remains strictly clinical.

Pediatric movement disorders are a heterogeneous group of disorders with varied etiologies. Although many movement disorders that present in adults can also present in infants and children, there is a subset of conditions that are unique to the pediatric population. One such broad category are the benign and developmental movement disorders of infancy and early childhood. In general, these movements disorders are transient, and outcomes are positive. Accurate diagnosis can minimize the need for additional testing and provide families with reassurance.

Although movement is largely generated from the primary motor cortex, what movement to make and how to make it is influenced from the entire brain. External influences from the environment come from sensory systems in the posterior part of the brain, and internal influences, such as homeostatic drive and reward, from the anterior part. A movement is voluntary when a person’s consciousness recognizes it to be so because of proper activation of the agency network. Behavioral movement disorders can be understood as dysfunction of these mechanisms. Apraxia and task specific dystonia arise from disruption of parietal–premotor connections. Tics arise from a hyperactive limbic system. Functional movement disorders may also have an origin in abnormal limbic function and are believed to be involuntary due to dysfunction of the agency network. In Parkinson’s disease, bradykinesia comes from insufficient basal ganglia support to the anterior part of the brain.

This chapter deals with abnormal, spontaneous and reactive motor behavior as part of the clinical expression of some psychiatric disorders, including abnormal motility, locomotion, gestures, mimic, and speech. Here, the differentiation of the abnormal motor behavior motor dysfunction as an integral part of a psychiatric condition or as a side effect of its treatment is critical for the management but often remains difficult to differentiate. Iatrogenic movement disorders, as might be seen in the treatment of specific psychiatric disorders, for instance with neuroleptics, are discussed in Chapter 51. In this chapter, we focus on the signs and symptoms of movement disorders as an integral, genuine part of the clinical manifestation, sometimes even in prodromal states, in psychiatric diseases, such as in schizophrenia, catatonia, and stereotypies, as well as in major depressive disorders, attention deficit hyperactivity disorders, obsessive-compulsive disorders, and impulse control disorders. Psychogenic (functional or somatoform) motor behavioral abnormalities, the result of conversion, somatization and/or factious disorders (malingering), are described in Chapter 53.

Despite increasing dilapidation, many of Rome’s ancient buildings survived in a form to impress visitors. During the Middle Ages a number of them – Hildebert of Lavardin, Master Gregorius, Benjamin of Tudela – left a brief record of the favourable impression the ruins made upon them. More widespread, however, were the legendary accounts, as found most extensively in the Mirabilia Urbis Romae, of the history and function of a number of the ruins of the pagan past. Such fables can be seen as forerunners of later ruin-mindedness in their attempt to explain the original role in the urban fabric of what was now ruinous and puzzling.

Dystonia is a heterogeneous group of diseases with important variability in phenomenology and underlying etiology and pathophysiology. Treatment must be individualized according to the symptomatology and needs of a specific patient. Several efficacious treatments to improve the symptoms of dystonia exist, but few treatments for metabolic and other disorders causing dystonia. The most important therapeutic options are described and discussed. Better understanding of the genetics and pathophysiology of dystonia, the progress of deep brain stimulation, and the possibility of physical therapy greatly improved multimodal therapeutic management of the dystonia patient. However, we are far from a cure; we can only rarely eliminate symptoms, with a few exceptions. For successful treatment, we need to consider specific motor and non-motor aspects of dystonia, different from other movement disorders. It is particularly important to understand the possibility and limitation of each therapeutic option in order to propose and combine different treatments according to the needs of a specific patient.

Myoclonus is defined as a sudden, brief, lightning-like involuntary movement that is caused by muscle contraction (positive myoclonus) or sudden inhibition of muscle contraction (negative myoclonus). It presents a challenging picture for several reasons. (1) There is a vast array of clinical presentations, which makes even the identification of myoclonus difficult. (2) Myoclonus is a clinical symptom, for which there are numerous causes. (3) There are multiple pathophysiologic mechanisms underlying myoclonus. For these reasons, there are several classification schemes and the differential diagnosis is broad. Further, treatment of myoclonus is hampered by a scarcity of large-scale randomized studies. As such, it is difficult to identify a unified and comprehensive approach to clinical evaluation and treatment of myoclonus. Although there is presently no consensus as to the ideal method of classifying or clinically approaching myoclonus, the various classification schemes each incorporate important aspects of myoclonus with the goal of devising an optimal treatment strategy.