Investigations Needed to Exclude

E-table 3

Etiology /

Investigations needed to exclude

Degenerative
spinocerebellar ataxia / patient history, family history, neurological examination, MRI, EMG and NCS, genetic testing
sporadic adult-onset ataxia / patient history, family history, neurological examination, MRI
multisystem atrophy / patient history, neurological examination, MRI, SPECT
Cerebellar ischemia / patient history (esp. cardiovascular risk factors), neurological and general clinical examination, MRI, MR angiography, doppler/duplex sonography, EKG, echocardiograph, laboratory testing (including HbA1c, serum lipids)
Craniocervical malformation / patient history, neurological examination, MRI
Toxic (amiodarone, carbamazepine, ethanol, lithium, opioids, phenytoin) / patient history, neurological examination, laboratory testing (including serum levels of suspected drug)
Inflammatory / infectious / patient history, neurological and general clinical examination, MRI, lumbar puncture, laboratory testing (including ANA, ANCA)
Neoplastic / paraneoplastic / patient history, general clinical and neurological examination, MRI, PET, whole-body CT scan, laboratory testing (including anti-neural antibodies, in particular anti-Purkinje cell antibodies)
Episodic ataxia type 2 / patient history, family history, neurological examination, genetic testing
Demyelinating disease / patient history, neurological examination, MRI, evoked potentials, lumbar puncture
Vestibular migraine / patient history, neurological examination, MRI, audiogram, ENG
Metabolic (vitamin B12 deficiency) / patient history, neurological and general clinical examination, EKG, laboratory testing
Hydrocephalus / increased intracranial pressure / patient history, neurological examination, MRI, lumbar puncture
Head trauma / patient history, neurological examination, MRI
Dolichoectasia of the vertebrobasilar artery / patient history, neurological examination, MRI, MR angiography, doppler/duplex sonography

E-table 3. The most common causes of "secondary" DBN and how they can be confirmed or excluded by patient history, family history, neurological and general clinical examinations as well as technical investigations. The etiologies are listed in order of decreasing frequencies, however, all causes listed below "neoplastic / paraneoplastic" are rare or have only been describe in single cases. While we have listed the most helpful diagnostic tools for each condition, it is necessary to adapt the tests to the individual patient (ANA = anti-nuclear antibodies, ANCA= anti-cytoplasmic antibodies, EKG= electrocardiogram, EMG and NCS= electromyography and nerve conduction studies, ENG= electronystagmography, MRI= magnetic resonance imaging, SPECT= single photon emission computed tomography).In part adapted from (6).