Cerebellar mutism.

Mutism of cerebellar origin is a well-described clinical entity that complicates operations for posterior fossa tumors, especially in children. This review focuses on the current understanding of principal pathophysiological aspects and risk factors, epidemiology, clinical characteristics, treatment strategies, and outcome considerations. The PubMed database was searched using the term cerebellar mutism and relevant definitions to identify publications in the English-language literature. Pertinent publications were selected from the reference lists of the previously identified articles. Over the last few years an increasing number of prospective studies and reviews have provided valuable information regarding the cerebellar mutism syndrome. Importantly, the clarification of principal terminology that surrounds the wide clinical spectrum of the syndrome results in more focused research and more effective identification of this entity. In children who undergo surgery for medulloblastoma the incidence of cerebellar mutism syndrome was reported to be 24%, and significant risk factors so far are brainstem involvement and midline location of the tumor. The dentate-thalamo-cortical tracts and lesions that affect their integrity are considered significant pathophysiological issues, especially the tract that originates in the right cerebellar hemisphere. Moderate and severe forms of the cerebellar mutism syndrome are the most frequent types during the initial presentation, and the overall neurocognitive outcome is not as favorable as thought in the earlier publications. Advanced neuroimaging techniques could contribute to identification of high-risk patients preoperatively and allow for more effective surgical planning that should focus on maximal tumor resection with minimal risk to important neural structures. Properly designed multicenter trials are needed to provide stronger evidence regarding effective prevention of cerebellar mutism and the best therapeutic approaches for such patients with a combination of pharmacological agents and multidisciplinary speech and behavior augmentation.

The death of Anna Katharina Ehmer: a case study in terminal lucidity.

Terminal lucidity, the unexpected return of mental clarity and memory shortly before the death of patients suffering from severe psychiatric and neurologic disorders, has raised the curiosity of numerous physicians and laypersons in the past. However, this peculiar phenomenon has received little attention in the recent decades. In previous publications, we have presented overviews of cases of terminal lucidity in various neurologic and psychiatric disorders. In this article, we highlight terminal lucidity in persons with mental disabilities, and focus on one of the most remarkable cases that have been reported. We provide biographical background information about the two principal witnesses who reported this case. It concerns the death of Anna Katharina Ehmer, a 26-year-old woman with severe mental disabilities who lived in an institution for people with mental disorders, and who had allegedly never spoken a single word during her life. Yet, she was reported to have sung dying songs for a half hour before she died. The case was reported by the head of this institution and by its chief physician. We consider it difficult to evaluate the authenticity of the case definitively in retrospect. Nevertheless, there are similar cases and a variety of other anomalous brain-related findings we consider worth investigating. Studies into such anomalous cases might improve our concepts of human brain functioning and of mental processing in persons with mental disabilities, and might be of special value for the dying, the bereaved, and caretakers.

'Study of several involuntary functions of the apparatus of movement, gripping, and voice' by Jean-Marc Gaspard Itard (1825).

Itard's 1825 paper, written while he was Chief Physician at the National Institute for Deaf-Mutes in Paris, demonstrates his empiricist approach to medicine. That is, Itard founded his medical practice on sense and experience rather than on surgery and medication. If all knowledge came through the senses, Itard reasoned, those lacking knowledge or social abilities could be improved by appropriate sensory stimulation. This concern with senses and society, along with his different approaches to men and women, his references to contemporary cures and his comparisons between humans and animals, document early nineteenth-century medical and psychological attitudes and treatments. Itard's paper also contains what was later recognized as the first clinical observation of Gilles de la Tourette Syndrome (TS).

Itard's 1828 mémoire on "Mutism caused by a lesion of the intellectual functions": a historical analysis.

OBJECTIVE: To demonstrate that Itard was one of the first clinicians to describe autism (which Itard named "intellectual mutism") and effectively separate these cases from mental retardation. Known for his attempts at rehabilitation of the Wild Boy of Aveyron, Itard wrote a largely unacknowledged paper in 1828 on the different causes of "intellectual mutism," the result of 28 years of observations at the Institut des Sourd-Muets in Paris. METHOD: Itard emphasized a complete examination of the child's faculties including attention, memory, and imitative capacity. He also described the behavior of these children as unsocialized, with poor peer relationships, superficial contact with adults in order to satisfy their own needs, and difficulties in language, especially with pronouns. RESULTS: He then described his various diagnostic and treatment approaches to determine whether the child can regain language and is educable. His description of the key features of intellectual mutism is compared to Kanner's classic description of autism. CONCLUSION: Itard rejected the overly inclusive diagnosis of "idiocy" and offered a way to distinguish children with mental retardation from those with pervasive developmental disorders, described key clinical features, and offered an assessment and treatment of these cases, all before 1830. His contribution should be recognized in textbooks of child psychiatry and developmental disorders.

Behaviour modification by successive approximation: Saxon age examples from Bede.

This paper describes the treatment of a case of mutism in Saxon England. The shaping procedure employed was similar to that reported in modern behaviour modification studies. The paper quotes from a seventh century papal letter showing an understanding of the principle of shaping as applied to populations.