[A description of early hydrocephalus with macrocephaly, cerebral palsy, epilepsy, intellectual disability, and growth retardation by Norwegian novelist Amalie Skram (1846-1905)]. / Hans (Hansemann) Tønnesen, en diagnostisk gåte fra Amalie Skram.

In her novel «S.G. Myre¼ written in 1890, Norwegian author Amalie Skram describes Hans (Hansemann) Tønnesen, a 16-year-old boy with an enormous head, convulsions, and a rudimentary command of language. He is bullied by children in the street and is held in low esteem by his mother, who refers to him as a beast, crazy, or «a cross¼. Presumably, the boy has hydrocephalus with macrocephaly, epilepsy, and mental retardation. His retarded growth, small teeth and cracked skin suggest growth hormone deficiency resulting from his hydrocephalus. A couple of interesting details, such as his «splaying his fingers and moving them in various directions to make himself understood¼ and his «becoming red with exertion and grimacing terribly¼ when trying to speak, suggest extrapyramidal, dyskinetic cerebral palsy. He may also be deaf. A cousin of Skram's shared several of these traits and is a likely model for Hansemann. A question that occurs to a modern reader is to what degree Hansemann is mentally retarded, or whether he is wrongly perceived as such because of his physical challenges and his communication problems.

Skull deformations in craniosynostosis and endocrine disorders: morphological and tomographic analysis of the skull from the crypt of the Silesian Piasts in Brzeg (16th-17th century), Poland.

RESULTS: of morphological and tomographic (CT) studies of the skull that was found in the crypt of the Silesian Piasts in the St. Jadwiga church in Brzeg (Silesia, Poland) are presented and discussed here. The established date of burial of probably a 20-30 years old male was 16th-17th century. The analyzed skull showed premature obliteration of the major skull sutures. It resulted in the braincase deformation, similar to the forms found in oxycephaly and microcephaly. Tomographic analysis revealed gross pathology. Signs of increased intracranial pressure, basilar invagination and hypoplasia of the occipital bone were observed. Those results suggested the occurrence of the very rare Arnold-Chiari syndrome. Lesions found in the sella turcica indicated the development of pituitary macroadenoma, which resulted in the occurrence of discreet features of acromegaly in the facial bones. The studied skull was characterized by a significantly smaller size of the neurocranium (horizontal circumference 471 mm, cranial capacity ∼ 1080 ml) and strongly expressed brachycephaly (cranial index=86.3), while its height remained within the range for non-deformed skulls. A narrow face, high eye-sockets and prognathism were also observed. Signs of alveolar process hypertrophy with rotation and displacement of the teeth were noted. The skull showed significant morphological differences compared to both normal and other pathological skulls such as those with pituitary gigantism, scaphocephaly and microcephaly.

Craniofacial configuration and postcranial development of a hydrocephalic child (ca. 2500 B.C.-500 A.D.): with a review of cases and comment on diagnostic criteria.

Hydrocephalus is a severe disorder of the central nervous system characterized by absorption blockage of the cerebral spinal fluid (CSF). The archaeological record of the condition ranges in time from 10,000 B.C. to 1670 A.D. and consists of 30 possible cases worldwide. A review of this material reveals that diagnostic criteria which fully delineate the condition have not been established. Previously, no attempt has been made to differentiate the two major categories of hydrocephalus and their subgroupings, or to identify other conditions which might result in similar morphologies. A partial child's skeleton from the Middle Period (ca. 2500 B.C. to 500 A.D.) of Central California Prehistory is described in light of an extensive clinical literature. Examination of this individual reveals a unique craniofacial configuration and malformed postcrania. Bony criteria for a differential diagnosis of hydrocephalus are established and applied to this individual. Based on these criteria, the individual is diagnosed as having a chronic form of noncommunicating hydrocephalus. Blockage of the CSF pathway most likely occurred in the aqueduct of Sylvius with a partial occlusion of the foramen of Monro or a frontal cyst. In addition, femoral development is suggestive of partial paralysis.