Table 2.

Clinical features of CLIPPERS^*.

Symptoms/signs referable to brainstem-, cranial nerve-and/or cerebellar dysfunctions

Ataxia (gait ataxia, stance ataxia, truncal ataxia, limb ataxia) Dysarthria Dysphagia Dysgeusia Diplopia/oculomotor abnormalities (oculomotor palsies, gaze palsy, internuclear ophthalmoplegia, one-and-a-half syndrome, disturbances of saccadic eye and slow eye pursuit) Nystagmus (spontaneous, gaze evoked, upbeat, downbeat, rotational nystagmus) Altered sensation or tingling of the face (facial tingling, par-/dysaesthesias, hypaesthesia), altered sensation of the scalp, palate or tongue Facial nerve palsy Vertigo, hyperacusis, hearing impairment, tinnitus Hoarse voice Tongue weakness Hiccup Nausea

Symptoms/signs referable to long tract affections and/or spinal cord syndrome

Paraparesis, tetraparesis, hemiparesis, paresis of a single extremity Spasticity, long tract motor signs (extensor plantar response, hyperreflexia) Altered sensation/sensory loss of extremities (bilateral, unilateral; hemi-hypaesthesia, tetra-hypaesthesia, hypoaesthesia in single limbs) Decreased vibration sense Neurogenic bladder (urine retention/incontinence)

Cognitive dysfunction

Cognitive deficits, dysexecutive syndrome, psychomotor slowing Frontal disinhibition reflexes

Possible additional features

Pseudobulbar affect (pathological/involuntary crying or laughter, labile affect) Tremor (action, intention, Holmes tremor) Headaches Abnormal fatigue

Generally absent symptoms/signs

Quantitative consciousness Fever, night sweating, weight loss Lymphadenopathy Arthritis Uveitis Oral and/or genital ulcers; pathergia Sicca syndrome (keratoconjunctivitis sicca, xerophthalmia), Meningism Altered symptoms of hypothalamic dysfunction (polydipsia/ polyuria)

^*Symptoms/signs marked in italic type are encountered prevalently in CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) patients.