Signal reduction in the globus pallidus, which probably is due to abnormal accumulation of iron, is severe in PKAN and can be differentiated from normal with high reliability. The substantia nigra is affected to a lesser degree, and the red nucleus is not involved. The reason for this selective susceptibility of normally iron-rich brain structures for pathological accumulation of iron remains speculative. Our quantitative results might be helpful to assess the value of an iron chelation approach to therapy.

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SUMMARY -The case presented describes the simultaneous occurrence of an unusually large anterior fontanellar bone with a syndrome of vascular malformation and overgrowth in a three-monthold child, which to our knowledge has not yet been reported. This combination may strengthen the arguments for a possible genetic contribution to the occurrence of supernumerary ossicles in the skull. Although of minor clinical importance, the shape and variations of these Wormian bones should be well-known to prevent misleading interpretations of imaging Results.

Background and Importance Transtegmental brain herniation into the petrous bone is a rare cause of rhinoliquorrhea. Our case presents a combination of several typical clinical and imaging findings illustrating the ongoing etiologic discussion of such cerebrospinal fluid (CSF) fistulas. Clinical Presentation A 53-year-old man presented with nasal discharge after a strong effort to suppress coughing. Imaging revealed a transtegmental herniation of parts of the inferior temporal gyrus into the petrous bone and in addition a combination of signs of chronically increased intracranial pressure and a hyperpneumatization of the petrous bone. The fistula was closed by a middle cranial fossa approach. Conclusion The case illustrates the two main predisposing factors for development of petrous bone CSF fistulas: increased intracranial pressure and thinning of the tegmental roof due to extensive development of air cells. Because the CSF leakage repair does not change the underlying cause, patients have to be informed about the possibility of developing increased intracranial pressure and recurrences of brain herniations at other sites.