Monocentric study of 28 cases of chronic inflammatory demyelinating polyneuropathy: first Tunisian study.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare affection of the peripheral nervous system. Its diagnostic criteria have evolved since 1975. The aim of our work is to study the epidemiological, clinical, and paraclinical aspects of CIDP. METHODS: We conducted a retrospective study of 28 CIDP patients of the neurology department of the military hospital of Tunis between January 2000 and December 2017. All these patients met the European Federation of Neurological Societies/Peripheral Nerve Society(EFNS/PNS)2010 diagnostic criteria for definite CIDP. RESULTS: The average age was 50 years with a gender ratio of 1.57. We found sensitivomotor symptoms in 66% of patients. Neurological assessment showed a proximal and distal motor weakness in 50% of cases, the involvement of superficial and deep sensory systems in 44% of patients with a generalized areflexia in all patients. Median Inflammatory Neuropthy Cause and Treatment (INCAT) score was 7. Concerning electrophysiology, all our patients met the EFNS/EPS 2010 diagnostic criteria for a definite CIDP. Screening for concurrent pathologies was positive in 11 patients. On the therapeutic side, there was no superiority of intravenous immunoglobin compared with pulsed methylprednisolone. Oral steroids were used as backup in about 50% of patients. There were good outcomes in 72% of patients who improved very well after treatment. CONCLUSION: CIDP is a rare and polymorphic disorder with a variety of concurrent pathologies. Our study is the first study in Tunisia and in Maghreb countries which included the most big series of patients. Our results were similar to literature. A multicentral study would be better profitable.

Association between an angiotensin-converting enzyme gene polymorphism and Alzheimer's disease in a Tunisian population.

BACKGROUND: The angiotensin-converting enzyme gene (ACE) insertion/deletion (I/D or indel) polymorphism has long been linked to Alzheimer's disease (AD), but the interpretation of established data remains controversial. The aim of this study was to determine whether the angiotensin-converting enzyme is associated with the risk of Alzheimer's disease in Tunisian patients. METHODS: We analyzed the genotype and allele frequency distribution of the ACE I/D gene polymorphism in 60 Tunisian AD patients and 120 healthy controls. RESULTS: There is a significantly increased risk of AD in carriers of the D/D genotype (51.67% in patients vs. 31.67% in controls; p = .008, OR = 2.32). The D allele was also more frequently found in patients compared with controls (71.67% vs. 56.25%; p = .003, OR = 2.0). Moreover, as assessed by the Mini-Mental State Examination, patient D/D carriers were more frequently found to score in the severe category of dementia (65%) as compared to the moderate category (32%) or mild category (3%). CONCLUSIONS: The D/D genotype and D allele of the ACE I/D polymorphism were associated with an increased risk in the development of AD in a Tunisian population. Furthermore, at the time of patient evaluation (average age 75 years), patients suffering with severe dementia were found predominantly in D/D carriers and, conversely, the D/D genotype and D allele were more frequently found in AD patients with severe dementia. These preliminary exploratory results should be confirmed in larger studies and further work is required to explore and interpret possible alternative findings in diverse populations.

Parsonage-Turner syndrome of the brachial plexus secondary to COVID-19 vaccine: A case report.

Parsonage-Turner syndrome (PTS) is a peripheral inflammatory neuropathy of unknown etiology. We present a rare case of a 50-year-old male patient with PTS post-COVID-19 BNT162b2 mRNA vaccine. Symptoms occurred 15 days after the second dose. He was treated with corticosteroids, analgesics, and physical rehabilitation with a partial recovery.

Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerves and respiratory tract involvement: A case report.

Sixteen percent of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely like acute idiopathic demyelinating polyneuropathy (AIDP) the demyelinating form of GBS, developing in <8 weeks 2. This entity is classified as acute-onset CIDP (A-CIDP) which presents overlapping clinical and electrophysiological findings with GBS during early stages of disease, but followed with a chronic course beyond 2 months. Also, those who have three or more treatment-related fluctuations (TRF) are included under this term. Distinguishing between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and acute idiopathic demyelinating polyneuropathy (AIDP) may be difficult during early stages but is crucial in order to guide treatment strategies without delay. These two forms share some overlapping clinical and electrophysiological findings, including some severe clinical features such as cranial nerve and respiratory tract involvement making the diagnosis of A-CIDP more difficult.

Neuro-meningeal cryptococcal infection revealing a multiple myeloma.

Rare cases of Cryptococcus have been documented in patients living with multiple myeloma. To date there has been no documented evidence of cryptococcosis revealing multiple myeloma. We reported a 63-year-old man who had a 2-months history continuous holocranial headaches, morning vomiting, complaining of blurred vision and fever. The biologic and the imaging showed a Cryptococcus meningoencephalitis. The search for a cause of immunodeficiency revealed a multiple myeloma. The diagnosis for Cryptococcus was confirmed according to an India ink stain, blood and cerebrospinal fluid culture. The patient's treatment for multiple myeloma was initiated with a chemotherapy regimen. The evolution was good without complication. Cryptococcosis, especially in the neuro-meningeal form, is a serious, deadly opportunistic infection. The search of an underlining immunodeficiency must be systematic. In this case, it was associated with early stage multiple myeloma.

The Plasminogen Activator Inhibitor 1 4G/5G Polymorphism and the Risk of Alzheimer's Disease.

OBJECTIVE: The aim of this study was to determine whether plasminogen activator inhibitor 1 (PAI-1) is associated with the risk of Alzheimer's disease (AD) in Tunisian patients. DESIGN AND METHODS: We analyzed the genotype and allele frequency distribution of the PAI-1 polymorphism in 60 Tunisian patients with AD and 120 healthy controls. RESULTS: The results show a significantly increased risk of AD in carriers of the 4G/4G and 4G/5G genotypes versus the wild-type 5G/5G genotype (4G/4G: 28.33% in patients vs 10.0% in controls; P < 10-3; OR = 8.78; 4G/5G: 55.0% in patients vs 38.33% in controls; OR = 4.45; P < 10-3). The 4G allele was also more frequently found in patients compared with controls; P < 10-3; OR = 3.07. For all participants and by gender, homozygotic carriers (4G/4G) were at an increased risk of AD over heterozygotes and women were at an increased risk over their male genotype counterparts. The odds ratio for AD among 4G/4G carriers for any group was approximately twice that of heterozygotes in the same group. Women homozygotes ranked highest for AD risk (OR = 20.8) and, in fact, women heterozygotes (OR = 9.03) ranked higher for risk than male homozygotes (OR = 6.12). CONCLUSION: These preliminary exploratory results should be confirmed in a larger study.

Recurrent status epilepticus in posterior reversible encephalopathy syndrome as initial feature of pediatric lupus: A newly diagnosed case and literature review.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with several predisposing conditions. Systemic lupus erythematosus (SLE), beginning in 15-20% in childhood, is considered as a potential underlying etiology of PRES. In children, status epilepticus (SE) rarely complicates PRES, and exceptionally occurs in SLE. METHODS: We report on an illustrative case of PRES complicating pediatric lupus revealed by recurrent SE, and we further review through a Pubmed search the previously reported cases of pediatric SLE, PRES and SE. RESULTS: We describe the case of a 12-year old girl who presented with recurrent status epilepticus associated to high blood pressure and renal involvement. Brain imaging showed classical aspects of PRES. Immunological tests including antinuclear, anti-DNA, and anticardiolipin antibodies were positive. The diagnosis of SLE was established. The Pubmed search identified a total number of 9 children with SE in SLE, and 26 with PRES, including our patient. CONCLUSIONS: We discussed the clinical and paraclinical features of PRES in SLE with epilepsy, their underlying pathophysiological aspects, and their management challenges. PRES should be considered in initial recurrent SE in children, justifying a battery of tests comprising immunological testing. Anticardiolipin antibodies seem to play a crucial role in epilepsy, PRES and renal involvement in pediatric SLE. Further studies are needed to clarify whether PRES should be considered one of the neuropsychiatric manifestations of SLE or a consequence of active disease in other organ systems or its treatment.

Association of HLA-DR/DQ polymorphism with Alzheimer's disease.

BACKGROUND: Alzheimer's disease (AD) is a complex disorder, resulting from an interaction between environmental and genetic factors. Several studies have addressed the association of AD with major histocompatibility complex (MHC) polymorphisms without arriving at any definite conclusions. The human leukocyte antigen (HLA) region is the key susceptibility locus in many immunological diseases. The aim of this study was to investigate the probable association between HLA-DR/DQ alleles and AD in Tunisian patients. METHODS: HLA-DR/DQ genotyping was performed using polymerase chain reaction sequence-specific primers with 55 AD patients and 100 healthy individuals serving as the control group. RESULTS: AD in Tunisian patients was found to be associated with the following alleles (Pc denotes Bonferroni corrected probability values): HLA-DRB1*15 (Pc < 10-3), DRB1*04 (Pc = 0.03) and DQB1*06 (Pc < 10-3). Two haplotypes found to be associated with the disease were DRB1*1501/DQB1*0602 (Pc < 10-3) and DRB1*0402/DQB1*0302 (Pc = 0.02). CONCLUSIONS: The authors believe this to be the first research linking the haplotypes DRB1*1501/DQB1*0602 and DRB1*04/DQB1*0302 with AD. Larger studies in other populations will be important to support the present findings of the possible susceptible risk of HLA-DR/DQ in AD.