Neuropathology
Microscopic features of PKAN include high levels of iron in the globus pallidus and the pars reticulata of substantia nigra, evident as a characteristic rust-brown discoloration in a pattern called the eye-of-the-tiger sign; lipofuscin and neuromelanin concentrated in the iron-accumulating areas; oval, nonnucleated structures representing swollen axons whose cytoplasm swells with vacuoles, referred to as spheroids, axon schollen, or neuroaxonal dystrophy; and Lewy bodies. Treatment
Phosphopantothenate has been shown to treat PKAN in a human, and also in a mouse model of the disease. Pantethine (a precursor of pantetheine) has been studied and shown to be effective in a mouse and in a fruit fly model of the disease. Prognosis
Survival rates for those diagnosed with typical PKAN, and left untreated is 11.18 years with a standard deviation of 7.8 years. A study reporting good outcomes in a single patient with late onset PKAN has been performed. Epidemiology
Prevalence data regarding this disorder remains incomplete, however it is estimated that anywhere between 1 in 1,000,000 to 3 in 1,000,000 individuals will be affected by this disorder (based upon observed cases in a population), but once again this is only an estimate as the disease is so rare it is difficult to statistically and accurately ascertain. History
PKAN was first described by Hallervorden and Spatz (1922). Their discovery was brought about by a diagnosis of a family of 12 in which five sisters exhibited progressively increasing dementia and dysarthria. Autopsies revealed brown discolorations in different areas of the brain (particularly of interest were the globus pallidus and substantia nigra regions).
The first digit is the thumb, followed by index finger, middle finger, ring finger, and little finger or pinkie. According to different definitions, the thumb can be called a finger, or not. English dictionaries describe finger as meaning either one of the five digits including the thumb, or one of the four excluding the thumb (in which case they are numbered from 1 to 4 starting with the index finger closest to the thumb). Anatomy
Skeleton
The thumb (connected to the trapezium) is located on one of the sides, parallel to the arm. The palm has five bones known as metacarpal bones, one to each of the five digits. Human hands contain fourteen digital bones, also called phalanges, or phalanx bones: two in the thumb (the thumb has no middle phalanx) and three in each of the four fingers. These are the distal phalanx, carrying the nail, the middle phalanx, and the proximal phalanx. Joints are formed wherever two or more of these bones meet. Each of the fingers has three joints:

metacarpophalangeal joint (MCP) – the joint at the base of the finger
proximal interphalangeal joint (PIP) –  the joint in the middle of the finger
distal interphalangeal joint (DIP)  – the joint closest to the fingertip.Sesamoid bones are small ossified nodes embedded in the tendons to provide extra leverage and reduce pressure on the underlying tissue. Many exist around the palm at the bases of the digits; the exact number varies between different people.