Neuronal intranuclear inclusion disease (NIID) is considered as a heterogeneous disease due to its various clinical symptoms including dementia, limb weakness, cerebellar ataxia,, parkinsonism, seizures, and autonomic dysfunction. Hemiplegic migraine with NIID has been rarely reported, and patients with migraine like attack as the only dominant symptom of NIID has not been reported. Here, we presented a NIID case of a young man suffered by sole symptoms mimic migraine with aura who was misdiagnosed as Susac syndrome in local hospital.

We report this NIID case with atypical symptom of migraine-like attack with aura. NIID should be considered as a differential diagnosis when a patient presents with migraine-like attack, persist DWI high intensity signals within white matter and early-onset brain atrophy which is not matched with age. Timely gene analysis and skin biopsy are helpful for definite diagnosis.