<unk>  presents itself in two ways: *<unk>  is a cortical degeneration that resembles amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration *<unk>  is a subcortical degeneration resembling parkinsonism and progressive supranuclear palsy.<unk>  As with<unk> , the symptoms and forms of<unk>  present themselves differently from patient to patient. Patient presentations include muscle atrophy, maxillofacial paralysis, inability to speak or swallow and subsequent choking. Some patients retain mental lucidity throughout the illness until death, much like ALS patients. Diaphragm and respiratory accessory muscles can become paralyzed necessitating mechanical ventilation to facilitate breathing. Saliva must be suctioned from the mouth to prevent aspiration. This form of<unk><unk>  is fatal in all cases.<unk>  No standard form of<unk>  has been reported and the documented cases of the disease manifested in many different clinical presentations. The doctor visited a patient who had just suddenly come down with a virulent form. His symptoms had begun 18 months before, starting with a strange immobility and a loss of initiative and spontaneity; he found he had to make a huge effort to walk, to stand, and to make the least movement—his body was disobedient. The immobility attacked with frightening speed, and within a year, he was unable to stand alone and could not control his posture (2006). —Oliver Sacks, The Island of the Colorblind, Vintage Books, 1996 Progressive dementia is also characteristic of<unk> . Those who experience dementia are often aphasic and restless, and demonstrate irrational behavior, such as violence, and deep emotions at odd intervals. Patients experience manic highs and lows, giggling one minute and screaming the next. Patients in the most virulent stage present with mouths hanging open, with excessive salivation; their tongues hang motionless, rendering speech and swallowing impossible. The patient’s arms and legs become severely spastic and bent in immovable tension. The advanced progression presents as profound motionlessness, or catatonia, accompanied with tremors or rigidity. Except in cases with concurrent dementia, most patients are capable of lucid thought and speech throughout the disease’s physical progression.
Lytico-bodig disease
Crigler–Najjar syndrome
Nakajo syndrome
Degos disease
Fazio–Londe disease
Brill–Zinsser disease
Rhizomelic chondrodysplasia punctata
DeSanto-Shinawi syndrome