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.

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.

Stroke disclosing primary aldosteronism: Report on three cases and review of the literature.

OBJECTIVES: There is a growing evidence of increased risk of cerebrovascular events in primary aldosteronism (PA). Nevertheless, acute neurologic ailment as presenting feature of PA is uncommon. Our aim is to highlight the diagnosis challenges in stroke unmasking PA and to discuss the underlying physiopathology and management dilemmas. MATERIALS AND METHODS: We hereby describe three consecutive rare cases of stroke revealing PA. All patients had brain imaging and thorough biological and morphological assessment to rule out other etiologies of stroke. The diagnosis of primary aldosteronism was established according to the Endocrine Society Clinical Practice Guideline, with a review of the literature on the spectrum of neurologic manifestations in PA. RESULTS: We report on three cases, two women and a man, presenting with ischemic or hemorrhagic stroke, of early onset in two of them. All of the reported patients had hypertension and hypokaliemia. This association prompted the assessment of renin angiotensin aldosterone system (RAAS) disclosing PA, which was due to bilateral adenomas in the first one or bilateral adrenal hyperplasia in the two others. All patients refused the surgical option and received spironolactone with recurrence of stroke in one of them due to treatment incompliance. CONCLUSION: Although cerebrovascular events are quite common in PA, their occurrence as initial feature can be misleading. The association of hypokaliemia and refractory hypertension in ischemic or hemorrhagic strokes should prompt an assessment of the RAAS to rule out PA and initiate adequate management as soon as possible in order to avoid further complications.

Occipital Sinus Thrombosis: An Exceptional Case Report.

BACKGROUND: Variations of the dural venous sinuses may result in inaccurate imaging interpretation or complications during surgical approaches. One variation of the dural venous sinuses reported infrequently in the literature is the occipital sinus. We report an exceptional case of occipital sinus thrombosis. CASE REPORT: A 48-year-old right-handed man with a 5-month history of hypertension and chronic renal failure presented with cephalalgia, vomiting, and blurred vision evolving over 48 hours. Neurological examination revealed papillary edema stage 1 with no others abnormalities. An initial brain computed tomography (CT) scan performed was normal. The opening pressure of cerebrospinal fluid (CSF) was 35 cmH2O with normal level of protein and no hypercellularity in CSF analysis. The evolution was marked by the occurrence of generalized tonic-clonic seizure. A second CT scan performed showed a hyperdensity of the occipital sinus. Magnetic resonance imaging and magnetic resonance venography studies confirmed the diagnosis with highlighting the thrombosis of the occipital sinus in association to an ectasia of the torcular. The patient received adequate anticoagulation for 6 months in association to antiepileptic drugs with a good evolution. DISCUSSION: According to our review, such a thrombosis must be a rare condition, because our literature search has shown a lack of any report describing this condition. Herein, we review the anatomy of the occipital sinus and we illustrate the characteristics of this unusual thrombosis with multiple imaging modalities. CONCLUSION: Understanding of the cerebral venous anatomy and recognition of venous variations essentially help when dealing with a pathology, which presents along with a particular venous variation, no matter how rare this combination is.