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[Neuronal intranuclear inclusion disease with prolonged impaired consciousness and status epilepticus: a case report].
Rinsho Shinkeigaku. 2019 Jul 31; 59(7):425-430.RS

Abstract

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease presenting with various manifestations including dementia, weakness, transient impaired consciousness, encephalitis-like episodes and also epileptic seizures. However, the nature of epileptic seizures, focal or generalized onset, remains unclear. A man at age 76 was admitted to a local hospital due to febrile impaired consciousness lasting several days. During the hospital stay, a generalized convulsion occurred, and afterward he remained obtunded. He was transferred to our hospital for further treatment. One additional seizure occurred while on an ambulance to our hospital and two additional seizures shortly after the arrival, which indicated convulsive status epilepticus (SE). The ictal EEG showed low amplitude fast activity arising from the left centro-parietal area with an evolutionary pattern. The clinical concomitant was the contralateral versive seizure evolving to a bilateral convulsion. Inter-ictal epileptiform abnormalities seen on the tracings taken on later days consisted of brief potentially ictal rhythmic discharges (B(I)RDs) and frequent sharp waves recorded from both frontal areas. These findings along with the ictal discharges would indicate a multifocal epileptic disorder in this patient. Diffusion weighted images (DWIs) of this patient showed hyperintensity signals in the cortico-medullary junctions in the bilateral frontal and the left parietal regions. Skin biopsy revealed characteristic intranuclear inclusions, and hence the diagnosis of NIID was made. The regions of epileptic foci on EEG corresponded well to the hyperintensity areas in DWIs, which suggests the cerebral cortices near the DWI hyperintensity could be more hyperactive than other regions. This case report suggests that some of the prolonged impaired consciousness and the encephalitis-like episodes in NIID could be related to epileptic seizures and even non-convulsive SE or a post-ictal twilight state after an unwitnessed convulsion. This issue should be further studied for the appropriate treatment of episodic encephalopathy and epileptic seizures in NIID.

Authors+Show Affiliations

Department of Neurology, Tenri Hospital.Shirakawa Branch, Tenri Hospital.Department of Neurology, Tenri Hospital.

Pub Type(s)

Case Reports
Journal Article

Language

jpn

PubMed ID

31243248

Citation

Yamanaka, Haruo, et al. "[Neuronal Intranuclear Inclusion Disease With Prolonged Impaired Consciousness and Status Epilepticus: a Case Report]." Rinsho Shinkeigaku = Clinical Neurology, vol. 59, no. 7, 2019, pp. 425-430.
Yamanaka H, Hashimoto S, Suenaga T. [Neuronal intranuclear inclusion disease with prolonged impaired consciousness and status epilepticus: a case report]. Rinsho Shinkeigaku. 2019;59(7):425-430.
Yamanaka, H., Hashimoto, S., & Suenaga, T. (2019). [Neuronal intranuclear inclusion disease with prolonged impaired consciousness and status epilepticus: a case report]. Rinsho Shinkeigaku = Clinical Neurology, 59(7), 425-430. https://doi.org/10.5692/clinicalneurol.cn-001264
Yamanaka H, Hashimoto S, Suenaga T. [Neuronal Intranuclear Inclusion Disease With Prolonged Impaired Consciousness and Status Epilepticus: a Case Report]. Rinsho Shinkeigaku. 2019 Jul 31;59(7):425-430. PubMed PMID: 31243248.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Neuronal intranuclear inclusion disease with prolonged impaired consciousness and status epilepticus: a case report]. AU - Yamanaka,Haruo, AU - Hashimoto,Shuji, AU - Suenaga,Toshihiko, Y1 - 2019/06/27/ PY - 2019/6/28/pubmed PY - 2019/10/8/medline PY - 2019/6/28/entrez KW - convulsive status epilepticus KW - epileptic seizure KW - neuronal intranuclear inclusion disease KW - prolonged impaired consciousness KW - skin biopsy SP - 425 EP - 430 JF - Rinsho shinkeigaku = Clinical neurology JO - Rinsho Shinkeigaku VL - 59 IS - 7 N2 - Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease presenting with various manifestations including dementia, weakness, transient impaired consciousness, encephalitis-like episodes and also epileptic seizures. However, the nature of epileptic seizures, focal or generalized onset, remains unclear. A man at age 76 was admitted to a local hospital due to febrile impaired consciousness lasting several days. During the hospital stay, a generalized convulsion occurred, and afterward he remained obtunded. He was transferred to our hospital for further treatment. One additional seizure occurred while on an ambulance to our hospital and two additional seizures shortly after the arrival, which indicated convulsive status epilepticus (SE). The ictal EEG showed low amplitude fast activity arising from the left centro-parietal area with an evolutionary pattern. The clinical concomitant was the contralateral versive seizure evolving to a bilateral convulsion. Inter-ictal epileptiform abnormalities seen on the tracings taken on later days consisted of brief potentially ictal rhythmic discharges (B(I)RDs) and frequent sharp waves recorded from both frontal areas. These findings along with the ictal discharges would indicate a multifocal epileptic disorder in this patient. Diffusion weighted images (DWIs) of this patient showed hyperintensity signals in the cortico-medullary junctions in the bilateral frontal and the left parietal regions. Skin biopsy revealed characteristic intranuclear inclusions, and hence the diagnosis of NIID was made. The regions of epileptic foci on EEG corresponded well to the hyperintensity areas in DWIs, which suggests the cerebral cortices near the DWI hyperintensity could be more hyperactive than other regions. This case report suggests that some of the prolonged impaired consciousness and the encephalitis-like episodes in NIID could be related to epileptic seizures and even non-convulsive SE or a post-ictal twilight state after an unwitnessed convulsion. This issue should be further studied for the appropriate treatment of episodic encephalopathy and epileptic seizures in NIID. SN - 1882-0654 UR - https://www.unboundmedicine.com/medline/citation/31243248/[Neuronal_intranuclear_inclusion_disease_with_prolonged_impaired_consciousness_and_status_epilepticus:_a_case_report]_ L2 - https://dx.doi.org/10.5692/clinicalneurol.cn-001264 DB - PRIME DP - Unbound Medicine ER -