Table 1.
ID | Diagnosis | Antibodies | Age at diagnosis, sex | Time first symptoms to diagnosis/VOG | Initial symptoms | Symptoms at last follow-up (FU) visit | cMRI | Tumor | mRS | ||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Sleep disorder | Movement disorder | Cognition | Bulbar dysfunction | Other | y/n | Last FU | |||||||
Pat.1 | Anti-IgLON5 disease | S 1:400 C 1:256 |
64, f | 2 /5 years | cognitive dysfunction, epileptic seizures | #, + | – | + | – | da, pnp | ha (b) | n | 2 |
Pat. 2 | Anti-IgLON5 disease | S 1:3200 C 1:16 |
72, f | 5 /5 years | stridor, vocal cord palsy | #, + | + | + | + | da | le | n | 4 |
Pat. 3 | Anti-IgLON5 disease | S 1:12.800 C 1:128 |
77, f | 10 /10 years | vertigo, gait unsteadiness | #, + | + | + | + | pnp | le | y | 6 |
Pat. 4 | Anti-IgLON5 disease | No titer available | unknown, m | 14 (post mortem) /8 years | dysphagia, ataxia | #, + | + | + | + | pnp | iat | n | 6 |
Pat. 5 | PSP-RS | No | 70, m | 2 /2 years | dysarthria, falls, gait unsteadiness cognitive dysfunction, aphasia, tremor | #, + | + | + | + | da | le | n | 6 |
Pat. 6 | PSP-RS | No | 68, m | 8 / 10 years | gait unsteadiness, dysarthria, bradykinesia | *- | + | - | + | pnp | n.a. | n | 4 |
Pat. 7 | PSP-RS | No | 74, m | 3 /5 years | falls, bradykinesia, gait unsteadiness, rigor, dysarthria, dysphagia, extensive snoring | *+ | + | - | + | - | ga | n | 4 |
Pat. 8 | PSP-RS | No | 73, f | 1 /1 year | falls, gait unsteadiness, dysarthria, dysphagia | *- | + | - | + | - | le | n. | 6 |
Pat. 9 | PSP-RS | No | 67, f | 2 /2 years | limb ataxia, gait unsteadiness, dysarthria, apraxia | *- | + | - | + | - | ca, ma | n | 6 |
Pat. 10 | PSP-P | No | 66, f | 2.5 / 3 years | dysphagia, dysarthria, gait unsteadiness, falls, cognitive dysfunction | *- | + | + | + | - | ga, ma, cs, ha | n | 4 |
Pat. 11 | PSP-P | No | 70, f | 2 / 4 years | dysphagia, dysarthria, gait unsteadiness | *, + | + | + | + | - | ga | n | 5 |
Pat. 12 | PSP-P | No | 65, m | 1.5 / 2 years | rigor, gait unsteadiness | *, - | + | - | + | - | le | n | 4 |
Pat. 13 | PSP-P | No | 68, f | 1.5 / 2 years | supranuclear gaze palsy, gait unsteadiness | *, - | + | - | + | - | ta (b) | n | 4 |
Pat. 14 | PSP-P | No | 72, m | 1.5 / 2 years | gait unsteadiness, falls, bradykinesia | *, - | + | + | - | - | le | n | 3 |
Sleep disorder—confirmed by: video PSG #, by history*; movement disorder: parkinsonism, chorea, facial spasm, myoclonus, ataxia; bulbar dysfunction: dysphagia, dysarthria, stridor, vocal chord palsy, sialorrhea, respiratory failure; other: da, dysautonomia (excessive sweating, bradycardia/tachycardia), pnp, polyneuropathy; MRI: ha, hippocampal atrophy (u, unilateral; b, bilateral), ta- temporal atrophy (u, unilateral; b, bilateral), ca, cerebellar atrophy; ma, mesencephalic atrophy; cs, colibri sign; ga, generalized atrophy; le, leukoencephalopathy; iat, ischemic area thalamus; S, serum; C, cerebrospinal fluid