Table 4 Differential diagnosis of isolated neurological vertigo in adulthood

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]