Table 4.

Differential diagnosis of isolated neurological vertigo in adulthood

Differential Diagnosis	Incidence/Prevalence	Main Features	Clues for Differential	Examination Required	References
Primary or secondary brain tumours
Cerebellar lymphoma	CNS lymphoma represents 2–6% of all primary brain neoplasms (1.34 cases per million people); cerebellar involvement presents in only 9% of cases	Sudden onset of vertigo associated with vomiting	Neurotological evaluation: atypical nystagmus patterns during diagnostic maneuvers may raise suspicion of central pathology	Brain MRI with contrast enhancement and biopsy	Joshi et al., 2020 [22]
Cerebellar metastases	98,000–170,000 cases of brain metastases/year; metastases to the cerebellum accounts for 10–15% of all brain metastasis	Onset with severe headache, associated with nausea and vomiting, followed by positional vertigo and unsteady standing	Neurotological evaluation: atypical nystagmus patterns during diagnostic maneuvers may raise suspicion of central pathology	Brain MRI with contrast enhancement	Joshi et al., 2020 [22]
Infratentorial gliomas	Incidence of glioma is about 6.0 per 100,000 person-years; infratentorial gliomas represent 4.6% of all gliomas	Occasional attacks of vertigo and nausea lasting less than 30 seconds, related to changes in head position	Neurotological evaluation: atypical nystagmus patterns during diagnostic maneuvers may raise suspicion of central pathology	Brain MRI with contrast enhancement	Joshi et al., 2020 [22]
Ischemic stroke
Cerebellar stroke	2–3% of 600,000 stroke-year in the United States. Presumed stroke etiologies: atherosclerotic occlusive lesions of the vertebral artery (32%), in situ branch artery disease (25%), cardioembolism (10%), vertebral artery dissection (5%)	Sudden onset of rotational vertigo associated with neurovegetative symptoms (nausea and vomiting). Sometimes concomitant headache or unilateral hearing loss	Head Impulse Test (HIT) is positive in acute peripheral vertigo (APV) and negative in cerebellar strokes (pseudo-APV). Delayed onset of other central symptoms/signs is not uncommon	CT scan, MRI and neurotologic examination	Grad A et al. 1989 [23], Norrving et al. 1995 [24]; Kim GW et al. 1996 [25], Casani et al., 2013 [26], Joshi et al., 2020 [22], Doijiri et al., 2016 [27], Hesselbrock, 2017; Perloff et al., 2017 [28], Wang et al., 2018;
Pons stroke	7% of all ischemic strokes, 15–20% of posterior circulation ischemia. One in ten non-traumatic intracerebral hemorrhages is located in the pons	Vertigo and vomiting, falls and pointing towards the affected side, direction fixed nystagmus towards the unaffected side	Impairment of smooth pursuit eye movements may be present	MRI and neurotologic examination	Norrving et al. 1995 [24]; Kim GW et al. 1996 [25], Lee et al. 2009; Doijiri et al., 2016 [27], Wang et al., 2018
Medulla oblongata stroke	Not found exact incidence/prevalence. In a study: annual incidence of posterior circulation infarction is 18 per 100 000 person years in an Australian study (Dewey et al. 2003) 10–20% of them may cause acute vestibular syndrome	Diverse patterns of spontaneous nystagmus, gaze-evoked nystagmus and head-shaking nystagmus, possible otolithic dysfunction, subjective visual vertical (SVV) tilt, presence of at least one component of the ocular tilt reaction (OTR)	Less than a third of patients have abnormal ocular and cervical vestibular-evoked myogenic potentials (VEMPs) in lateral medullary infarction. Abnormal VEMPs are seen in about one-half of patients in medial medullary infarction	MRI and neurotologic examination	Paul et al., 2013; Sun-Uk Lee et al., 2015; Doijiri et al., 2016 [27], Wang et al., 2018
Persistent trigeminal artery (PTA)	Prevalence 0.1%-0.2% of cerebral angiograms	Isolated intermittent vertigo, followed by anterior and posterior circulation ischemic strokes symptoms	CT angiography evidence of PTA and CT signs of ischemic stroke	CT angiography	Parthasarathy, et al. 2016 [31]
Cephalalgia
Migraine	*The prevalence of migraine according to IHS criteria was higher in the isolated recurrent vertigo group (61.1%) than in the control group (10%; p < 0.01)	isolated recurrent vertigo of unknown cause	Extensive neurotological, including auditory and vestibular function testing and appropriate imaging studies	ICHD3 criteria	Lee et al., 2002 [32]
Demyelinating disorders
Multiple Sclerosis (MS) and Neuromyelitis Optica Spectrum Disorders (NMOSD)	The prevalence of MS in Europe is about 100–190/100.000 inhabitants; the prevalence range of NMOSD is ~ 0.5–4/100.000 worldwide	Isolated vertigo with or without nystagmus	Extensive neurotological, including auditory and vestibular function testing and MRI	Clinical exam, Brain MRI, HIT	Pula et al., 2013 [35], Kremer et al., 2014 [36]
Infectious
Neurocisticercosis	rare	Positional vertigo nystagmus	Cultural tests	Clinical exam, Brain MRI	Joshi et al., 2020 [22]
Cryptococcosis	rare	Fever, vertigo	Cultural tests	Clinical exama, Laboratory tests (CSF culture) neuroimaging (CT, MRI)	Adzic-Vukicevic et al., 2019[34]
Others
Vestibular neuritis	Unknown	Acute onset of vertigo with repetitive falls without hearing loss or tinnitus	recent viral infection	Serology for herpes virus	Lee JY et al., 2019 [37], Roberts RA et al., 2018 [38]
Arnold-Chiari malformation	Rare	Displacement of the cerebellar tonsils	Neuroradiology	Brain MRI	Unal M et al., 2006 [39]
Episodic ataxia type 2	Rare	Paroxysmal recurrent attacks of vertigo which usually respond to the treatment with potassium channel blockers and acetazolamide	autosomal dominant	Genetics	Spacey S et al., 1993 [44]
Hemiplegic migraine	Rare	Acute attack with isolated vertigo or more often associated with hemiparesis and confusion		Clinical exam, genetic testing	Rispoli et al., 2019 [40]
Bowhunter’s syndrome and	Very rare	Recurrent attacks of vertigo associated with neck rotation	Neuroradiology	Dynamic MRI and neurosonology	Di Stefano et al., 2020 [41]
Subclavian steal syndrome	Rare	Recurrent attacks of vertigo associated with the use of an arm	Neuroradiology	MRI and neurosonology	Potter et al., 2014 [42]
Cerebellar syndrome due to naturopathic over-the-counter supplements	Only a single report	Vertigo, gait unsteadiness, nystagmus, hypermetric saccades, dysmetria, ataxia	Anamnesis of supplement use	Clinical exam, Laboratory tests, Neuroimaging	Kim DD et al., 2019 [33]
Frontal lobe epilepsy	Rare	Seizures with onset from the frontal lobe	Antiepileptics (i.e., sodium valproate, levetiracetam, and lamotrigine)	EEG	Jiang et al., 2020 [43]