Seronegative acute encephalitis following COVID-19 vaccines: a case series of an overlooked diagnosis with literature review.

PURPOSE: Autoimmune encephalitis is a neurological emergency of new-onset altered mental status, caused by an exaggerated immune-mediated response that targets the central nervous system. Autoimmune encephalitis has become an emerging differential diagnosis, when a classical infection cannot explain neurological symptoms. Displaying overlapping clinical presentations, ranging from the insidious onset of cognitive deficiency to more severe forms of encephalopathy with refractory seizures, autoimmune encephalitis can be challenging for clinicians. When evidence of malignancy is absent and pathogenic autoantibodies are undetected, with typical clinical and imaging features of autoimmune encephalitis, seronegative autoimmune encephalitis may be considered. Recently, vaccination-related autoimmune encephalitis and acute encephalitis after COVID-19 vaccination have attracted attention. METHODS AND RESULTS: We report a case series consisting of three patients with autoimmune encephalitis occurring shortly after COVID-19 vaccination and a current review of all previous reported autoimmune encephalitis related to COVID-19 vaccines. CONCLUSION: We emphasise on the prompt diagnosis of autoimmune encephalitis induced by Covid-19 vaccines and its timely treatment to improve the clinical outcome of this severe neurological condition. Post-licencing vaccine safety surveillance for potential adverse events is essential for vaccine safety and public confidence.

External validations of the ABCD2 score in prediction of stroke risk after transient ischemic attack. A Tunisian hospital-based cohort study.

BACKGROUND AND PURPOSE: Identification of patients with high risk of stroke after transient ischemic attack (TIA) could be helpful to optimize stroke prevention. We aimed to externally validate the ABCD2 score for the prediction of stroke after TIA in a Tunisian population. METHODS: We conducted a retrospective observational study of consecutive patients admitted for TIA in four university hospitals in Tunisia. Patients were screened for onset of stroke. Sensitivity, specificity, positive and negative predictive values with areas under the receiver operating characteristic (ROC) curves were calculated for risk of stroke at 2, 7, 30 and 90 days after the index event. RESULTS: Of 415 patients screened in this study, the total cumulative subsequent stroke rates after TIA at 2, 7, 30 and 90 days were respectively, 4.8%, 10.6%, 13.5% and 20.2%. Using a cut-off value of 4, the ABCD2 showed an overall good sensitivity (95%, 97.7%, 96.4% and 97.6% respectively at 2, 7, 30 and 90 days). Areas under ROC cure of the ABCD2 score in patients with TIA for stroke onset at 2, 7, 30 and 90 days were respectively 0.67 (95% CI, 0.55-0.79), 0.79 (95% CI, 0.71-0.85), 0.79 (95% CI, 0.72-0.85), and 0.76 (95% CI, 0.70-0.81). CONCLUSION: Our findings suggest that the ABCD2 score could be used in our population to discriminate patient with TIA at low and high risk of developing recurrent stroke.

Mitochondrial DNA and Alzheimer's disease: a first case-control study of the Tunisian population.

BACKGROUND: Alzheimer's disease (AD) is the most common neurodegenerative disorder in humans and presents a major health problem throughout the world. The etiology of AD is complex, and many factors are implicated, including mitochondria. Mitochondrial alteration has been proposed as a possible cause of AD. Therefore, several studies have focused on finding an association between inherited mitochondrial DNA variants and AD onset. METHODS: In this study, we looked, for the first time, for a potential association between mitochondrial haplogroups or polymorphisms and AD in the Tunisian population. We also evaluated the distribution of the major genetic risk factor for AD, the apolipoprotein E epsilon 4 (APOE ε4), in this population. In total, 159 single-nucleotide polymorphisms (SNPs) of mitochondrial DNA haplogroups were genotyped in 254 individuals (58 patients and 196 controls). An additional genotyping of APOE ε4 was performed. RESULTS: No significant association between mitochondrial haplogroups and AD was found. However, two individual SNPs, A5656G (p = 0.03821, OR = 10.46) and A13759G (p = 0.03719, OR = 10.78), showed a significant association with AD. APOE 4 was confirmed as a risk factor for AD (p = 0.000014). CONCLUSION: Our findings may confirm the absence of a relation between mitochondrial haplogroups and AD and support the possible involvement of some inherited variants in the pathogenicity of AD.

A Case of Painful Diplopia after COVID-19 Vaccination: Could It Be Tolosa-Hunt Syndrome?

Herein, we report a rare case of Tolosa-Hunt syndrome (THS) following coronavirus disease 2019 (COVID-19) vaccine administration. A 64-year-old patient presented with recurrent horizontal diplopia and ipsilateral orbital pain, 2 weeks after being administered the COVID-19 vaccination. A diagnosis of THS was based on the relevant criteria after ruling out the differential diagnoses. The clinical presentation improved with corticosteroid administration. THS must be recognized as a complication of COVID-19 vaccination. This association can be explained by an autoimmune response.

Catatonia in patients with dementia: a case report.

Catatonia occurring as part of a clinical picture of dementia has been reported with almost all types of dementia. It remains under-diagnosed in older adults and those with dementia. We review a case of a young patient admitted in our psychiatric department for catatonia and after efficient treatment with Lorazepam, assessment revealed a dementia. Catatonia is a severe neuropsychiatric syndrome with an excellent prognosis if recognized and treated without delay.

Psychiatric disorders in the acoustic neuroma: about a case.

Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder characterized by formation of central nervous system tumors. They are associated to significant morbidity due to multiple problems such as hearing loss that can lead to many psychiatric disorders.

Primary Hyperparathyroidism and Hyperthyroidism in a Patient with Myotonic Dystrophy: A Case Report and Review of the Literature.

Various endocrine manifestations are commonly described in myotonic dystrophy (MD), including primary hypogonadism, diabetes mellitus, and thyroid and parathyroid dysfunction. We describe a 46-year-old woman with a family history of MD with her son. She was diagnosed with cardiac arrhythmia and required the implantation of a pacemaker. She was noted to have a bilateral cataract. She complained of muscle weakness, diffuse myalgia, and palpitation. The electromyography (EMG) showed myotonic discharges. Laboratory tests showed high serum calcium 2.83 mmol/L, serum phosphate 1.2 mmol/L, parathormone 362.5 pg/mL, thyroid stimulating hormone TSH 0.02 mIU/L (normal range: 0.34-5.6 mIU/L), FT4 21.17 ng/mL, and negative anti-thyroperoxidase antibodies. Cervical ultrasound revealed a multinodular goiter. The 99mTc-MIBI scintigraphy localized a lower right parathyroid adenoma. The clinical data, the family history of MD, EMG data, and endocrine disturbances were strongly suggestive of MD associated with hyperthyroidism and primary hyperparathyroidism.

Seizures and movement disorders induced by hyperglycemia without ketosis in elderly.

BACKGROUND: Non-ketotic hyperglycemia (NKHG) may increase the probability of seizures and movement disorders. METHODS: We describe a series of 14 elders admitted for seizures and movement disorders linked to NKHG. RESULTS: Twelve patients developed motor seizures and two others movement disorders. Glucose levels varied 9.28 to 32 mmol/l, while osmolarity values varied from 302.28 to 328 mosmol/l. All patients responded well to insulin therapy and four of them needed anti-epileptic drugs. CONCLUSION: Seizures or movement disorders in elderly with NKHG could be misdiagnosed as neurological diseases. Blood glucose must be audited whenever patients with seizures or movement disorders are encountered, as the condition may quickly resolve when NKHG is controlled.

Cerebral Aneurysms: A Rare Feature of Behçet's Disease-A Case Report and Review of the Literature.

Behçet's disease (BD) is a multisystem vascular inflammatory disease with several clinical manifestations. Intracranial aneurysms are an extremely rare but nevertheless severe complication of BD. We report a case of a 44-year-old man. The diagnosis of BD was made based on the presence of recurrent oral aphthous ulcers and positive human leukocyte antigen (HLA-) B51 in the absence of evidence of other diseases. MRI showed an ancient ischemic right capsulolenticular lesion, subacute white matter hypersignals of the left capsule lenticular region, and multiple arterial aneurysms. The patient underwent two-month systemic high-dose corticosteroids and immunosuppressive therapy associated with severe neurological deficiency upon admission and severe impairment upon discharge. A thorough review of the literature showed 20 case reports of intracranial aneurysms in BD.

48XXYY Syndrome in an Adult with Type 2 Diabetes Mellitus, Unilateral Renal Aplasia, and Pigmentary Retinitis.

A 45-year-old male was referred for diabetes mellitus. Clinical examination found a family history of multiple precocious deaths, strong consanguinity, personal history of seizures during childhood, small testicles, small penis, sparse body hair, long arms and legs, dysmorphic features, mental retardation, dysarthria, tremor, and mild gait ataxia. Investigations found pigmentary retinitis, metabolic syndrome, unilateral renal aplasia, and hypergonadotropic hypogonadism, and ruled out mitochondrial cytopathy and leucodystrophy. Karyotype study showed a 48XXYY chromosomal type. Renal aplasia and pigmentary retinitis have not been described in 48XXYY patients. They may be related to the chromosomal sex aneuploidy, or caused by other genetic aberrations in light of the high consanguinity rate in the patient's family.

Maternally inherited Leigh syndrome: T8993G mutation in a Tunisian family.

Leigh syndrome is a genetically heterogeneous, neurodegenerative disorder that predominantly affects children and leads to death within months or years. Mutations causing this disease have been found in both mitochondrial and nuclear DNA. The present report describes a Tunisian family with a maternally inherited Leigh syndrome harboring the mitochondrial T8993G mutation in the ATPase 6 gene. Polymerase chain reaction-restriction fragment length polymorphism analysis with the MspI restriction endonuclease, quantified with a digital image analyzer and gel documentation system, showed that the T8993G mutation was present with a high percentage in the blood of the three patients tested. This mutation was also detected in the asymptomatic mothers of these three patients (90, 96, and 60%). Two novel mitochondrial mutations were identified in the mitochondrial ATP6 gene -- T8741G (L72R) and A8795G (H90R) -- and three novel polymorphisms. Altogether, Leigh syndrome presenting the T8993G mutation in the ATPase 6 gene with variable heteroplasmic loads (44-98%) in a single Tunisian family is a novel finding.