Neuronal intranuclear inclusion disease presented with recurrent vestibular migraine-like attack: a case presentation

Danhua Zhao,Zhaoxia Wang,Sha Zhu,Jianwen Deng,Qinlan Xu,Xianzeng Liu

doi:10.1186/s12883-021-02367-6

Abstract

BackgroundNeuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder characterized by dementia, tremor, episodic encephalopathy and autonomic nervous dysfunction. To date, vestibular migraine (VM)-like attack has never been reported in cases with NIID. Here, we describe an 86-year-old patient with NIID who presented with recurrent vertigo associated with headache for more than 30 years.Case presentationAn 86-year-old Chinese woman with vertigo, headache, weakness of limbs, fever, and disturbance of consciousness was admitted to our hospital. She had suffered from recurrent vertigo associated with headache since her 50 s,followed by essential tremor and dementia. On this admission, brain magnetic resonance imaging revealed high intensity signals along the corticomedullary junction on diffusion weighted imaging (DWI). Peripheral neuropathy of the extremities was detected through electrophysiological studies. We diagnosed NIID after detecting eosinophilic intranuclear inclusions in the ductal epithelial cells of sweat glands and identifying an abnormal expansion of 81 GGC repeats in the 5’UTR of NOTCH2NLC gene.ConclusionsVM-like attack may be associated with NIID.

Highlights

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder characterized by dementia, tremor, episodic encephalopathy and autonomic nervous dysfunction
vestibular migraine (VM)-like attack may be associated with NIID
Neuronal intranuclear inclusion disease (NIID) is a clinically heterogeneous disorder mainly manifested with dementia, tremor, episodic encephalopathy, muscle weakness, and autonomic nervous dysfunction

Summary

Background

Vestibular migraine (VM) is an episodic disorder associated with both vestibular symptoms caused by migraine and the typical symptoms of migraine. Case report An 86-year-old Chinese woman admitted to our clinic, presenting with vertigo, headache, and vomiting for four days She successively developed weakness in the left limbs, the right limbs, fever, and disturbance of consciousness two days before admission. Repeated brain CT scans showed no abnormalities, resulting in the recommendation of painkillers and anti-vertigo medicines to alleviate symptoms In her 60 s, the patient gradually developed shaking of the hands when holding objects. Brain MRIs were performed, the results were unknown except for mild cerebral atrophy She had no known family history of neurological diseases. During the same follow-up period, the patient complained of episodic vertigo, headache, and vomiting (4 times, lasting for 2–3 days per time), but no fever or neurological dysfunctions were noticed

Discussion

Conclusions