[Rapidly progressing quadriparesis secondary to licorice (souss) intoxication]. / Tétraparésie rapidement progressive par intoxication à la réglisse (souss).

INTRODUCTION: Regular consumption of licorice based beverages can provoke pseudohyperaldosteronism with hypokalemia but can rarely lead to severe muscle weakness. CASE REPORT: A 62-year-old man was admitted for a one-week history of progressive weakness of the four limbs. Blood work revealed severe hypokalemia that was due to primary pseudo-hyperaldosteronism secondary to licorice intoxication. He became normal after correction of the electrolytes disturbances. DISCUSSION & CONCLUSION: In an area where a liquorice-based beverage (souss), is frequently and sometimes excessively consumed, pseudo-hyperaldosteronism secondary to licorice toxicity should be thought of in front of any muscle weakness accompanied by hypokalemia. A rapid correction of electrolytes disturbances leads to rapid improvement.

[Primary and secondary prevention of ischemic stroke]. / Prévention primaire et secondaire des accidents vasculaires ischémiques cérébraux.

Primary prevention is aimed at reducing the risk of stroke in asymptomatic people. The most effective prevention is through control of modifiable risk factors. Adequate blood pressure reduction, cessation of cigarette smoking and use of antithrombotic therapy in atrial fibrillation are the most effective measures. Carotid endarterectomy may be useful in selected patients. Although very useful for health in general, tight control of diabetes and hypercholesterolemia, physical exercise and alimentary diet did not show a major influence for primary stroke prevention. Aspirin seems to be not very effective for primary stroke prevention, whereas some ACE inhibitors (e.g. ramipril), ARBs (e.g. losartan) or statins, may have a preventive role beyond their antihypertensive or hypocholesterolemic properties. Secondary stroke prevention is aimed at reducing the risk of recurrence after a first stroke or transient ischemic attack. Acting on risk factors is probably as effective as in primary prevention. Carotid endarterectomy for symptomatic stenoses > 70% and anticoagulation in patients with atrial fibrillation are by far the most effective measures. Antiplatelet therapy (aspirin, ticlopidine, clopidogrel and long acting dipyridamole-aspirine association) reduces significantly stroke recurrence. Most recent data suggest also that perindopril, eprosartan and some statins are beneficial against stroke recurrence even in normotensive and normocholesterolemic patients.

[Anticholinergic syndrome after intoxication by lupine seeds (Tourmos)]. / Syndrome anticholinergique après intoxication par des graines de lupin (Tourmos).

INTRODUCTION: Yellow lupine seeds (Tourmos) are frequently used as snack in Lebanon but their potential toxicity is extremely rare (five published cases) and often undiagnosed. CASE REPORTS: Two patients presented with anticholinergic syndrome (mydriasis, mouth dryness, palpitations, general malaise) after ingestion of 200-500 g of bitter lupine seeds (not soaked enough in water). No other cause of intoxication was found, and symptoms disappeared spontaneously in 24-36h. DISCUSSION: Yellow lupine seeds need a long preparation (boiling then soaking with several changes of water) to debitter before consumption. The spontaneous bitter taste is mainly due to the presence of a toxic substance with anticholinergic properties, lupanine. An insufficient preparation or a preference for bitter lupin can result in intoxication.

Neurological disorders and travel.

Travel is associated with a number of neurological disorders that can be divided into two categories: (1) Neurological infections including encephalitides, neurotuberculosis, neurobrucellosis, cysticercosis and trichinosis. Some of these disorders can be prevented by vaccinations, such as Japanese B encephalitis and rabies, some by the use of insect repellents and some by avoiding raw milk products and undercooked meat. (2) Non-infective neurological disorders, such as acute mountain sickness and high altitude cerebral oedema, problems occurring during air travel such as syncope, seizures, strokes, nerve compression, barotrauma and vertigo, motion sickness and foodborne neurotoxic disorders such as ciguatera, shellfish poisoning and intoxication by cassava. This group of diseases and disorders could be prevented if the traveller knows about them, applies simple physiological rules, takes some specific medications and knows how to avoid intoxications in certain geographical areas. Meningococcal meningitis, malaria and jet lag syndrome are extensively discussed in other articles of this issue. The discussion in this paper will be limited to the other disorders.

Bilateral simultaneous hip fractures secondary to an epileptic seizure.

A 30-year-old man sustained bilateral simultaneous displaced subcapital fractures of neck of femur during an epileptic tonic-clonic seizure. After admission to the hospital approximately 18 hours later, internal fixation of the fractures with dynamic hip screw was undertaken. Post operatively, he was managed by early motion and weight bearing on the second day. Despite the severity of the fractures and delayed surgery, satisfactory union of the fractures was noted at 6 months when bone densitometry was normal. At 3 years follow up, there was no sign of avascular necrosis of the femoral heads.

Neuromuscular paralysis in the intensive care unit.

OBJECTIVE: To determine the features, causes, risk factors and outcome of acquired neuromuscular paralysis in critically ill patients. METHODS: Retrospective review of all confirmed cases of acquired polyneuropathy and myopathy examined by our Neurology service in the Intensive Care Unit (ICU), at King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia over a period of 5 years. All patients had comprehensive electrophysiological studies and one third had muscle and nerve biopsies. RESULTS: Thirty cases were included, 8 cases of polyneuropathy, 15 cases of myopathy and 7 cases of mixed neuropathy and myopathy. Absent deep tendon reflexes and absent sensory potential on nerve conduction studies were significantly suggestive of neuropathy. The level of creatine phosphokinase was not of great diagnostic value. Most polyneuropathy and myopathy cases had passed through a stormy ICU course with sepsis and multiorgan failure. The use of high doses of steroids was more associated with myopathy. Seven patients died in ICU, the others were discharged to the wards after a mean ventilation period of 40 days. One patient became chronic ventilator dependent. CONCLUSION: From this series and available literature, it seems that symptomatic myopathy is more frequent than polyneuropathy and some risk factors are common for both (sepsis and multiorgan failure) while the use of steroids is more associated with ICU myopathy. Treating sepsis and stopping corticosteroids results in the improvement of most of the cases.

Molecular analysis of the spinal muscular atrophy and neuronal apoptosis inhibitory protein genes in Saudi patients with spinal muscular atrophy.

OBJECTIVE: Spinal muscular atrophy (SMA) is a common, often fatal, autosomal recessive disease leading to progressive muscle wasting and paralysis as a result of degeneration of anterior horn cells of the spinal cord. The prevalence of SMA cases in the Kingdom of Saudi Arabia (KSA) is much higher than the European and North American population. Deletions or mutations in 2 genes, telomeric form of the survival motor neuron (SMN1) and the neuronal apoptosis inhibitory protein (NAIP), are known to be associated with SMA. The aim of this study is to examine the deletions or interruptions of the SMN1 and NAIP genes in Saudi patients. METHODS: The study included 121 Saudi SMA patients [type I (60 patients); type II (26 patients); and type III (35 patients)]. The deletions or interruptions of the SMN1 and NAIP genes were detected by using polymerase chain reaction. The study was carried out at the King Fahad National Guard Hospital, Riyadh, KSA between 2000 and 2002. RESULTS: The homozygous deletions of exons 7 and 8 of the SMN1 gene were found in 94% and 87% of the patients. Exon 5 of the NAIP gene was deleted in 70%, but its deletion was more frequent in SMA type I (93%) as compared to type II (54%) and type III (43%). Seven patients with SMA diagnosis did not show any of the above homozygous deletions. All 230 control subjects had at least one copy of both SMN1 and NAIP genes, as expected. CONCLUSION: Our results demonstrate that the deletion rate (94%) of the SMN1 gene in Saudi SMA patients is similar, irrespective of types, compared with patients of other ethnic groups. We also show that the incidence of NAIP deletion is higher in the more severe SMA cases and the dual deletion of the SMN1 and NAIP genes are more common in Saudi SMA type I patients compared with patients of other ethnic groups.

Deletion mutations in the dystrophin gene of Saudi patients with Duchenne and Becker muscular dystrophy.

OBJECTIVE: The deletion in the dystrophin gene has been reported for many ethnic groups, but until now the mutations in this gene have not been thoroughly investigated in Saudi patients with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). We examined the deletion pattern in the dystrophin gene of the Saudi patients applying multiplex-polymerase chain reaction (PCR). The aim of this study is to describe the outcome of our initial effort to identify mutations in the dystrophin gene in a representative group of Saudi patients with DMD and BMD. METHODS: Genomic deoxyribose nucleic acid was isolated from 41 patients with DMD and BMD (27 patients confirmed by muscle biopsy and 14 patients with clinical suspicion), 3 patients with limb girdle muscular dystrophy, 12 male relatives of the patients, and 5 healthy Saudi volunteers. A total of 25 exons around the deletion prone regions (hot spots) of the dystrophin gene were amplified. The study was carried out at the King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia between 2000 and 2002. RESULTS: The deletion of one or more exons was found in 21 of 27 DMD and BMD patients confirmed by muscle biopsy. The deletion in the gene was detected in 5 of 14 patients with DMD diagnosis, but not confirmed by dystrophin staining of muscle biopsy. No deletion in the dystrophin gene was detected in control Saudi volunteers, the limb girdle dystrophy patients, and the relatives of patients, as expected. CONCLUSION: The present study suggests that intragenic dystrophin gene deletions occur with the same frequency in Saudi patients compared with other ethnic groups. The PCR-based deletion analysis provides a reasonable first step in the diagnostic care of Saudi patients who may be afflicted with DMD and BMD.

A fatal complication of bronchiectasis. Brain abscess with intraventricular rupture.

The classic clinical manifestations of bronchiectasis are cough and daily production of purulent sputum for months to years. The most common complications are hemoptysis and respiratory failure. Brain abscess has become rare in the recent antibiotic era. In this report, we present a case of bronchiectasis complicated by brain abscesses. Despite the early diagnosis and appropriate management, and while the condition of the patient was improving, an intraventricular abscess rupture led to rapid coma then death. Presentation and management of this potentially fatal complication of bronchiectasis are discussed.

Diabetic neuropathy. Correlation with other diabetic microvascular complications.

OBJECTIVE: We studied the frequency of neuropathy in Saudi patients with definite diabetic microvascular complications and compared it to patients without complications. A high frequency of neuropathy in patients with definite microvascular complications would suggest a vascular etiology. METHODS: The study group consisted of 201 type-2 diabetic patients followed in the diabetic clinic of King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia. These patients were screened for microvascular disease. Only symmetrical distal sensory and motor neuropathy cases were included in the study. Screening for retinopathy was carried out according to Klien`s criteria and nephropathy was diagnosed if albuminuria, microalbuminuria, abnormal blood urea nitrogen or creatinine was present. RESULTS: There was a strong correlation between the prevalence of diabetic peripheral neuropathy, retinopathy (P<0.001) and nephropathy (P<0.01), in patients with type 2 diabetes mellitus. This strong correlation suggests a common underlying pathogenesis. CONCLUSION: We conclude that microangiopathy may be a major factor in the pathogenesis of diabetic neuropathy. Major risk factors for microangiopathy are the degree of glycemic control and duration of diabetes.

Solitary fibrous tumor of the scalp in a child.

Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that arise most commonly in the pleura but have been increasingly reported in extrapleural sites. The authors report the case of an SFT of the scalp that manifested as an enlarging scalp mass in a 2-year-old boy. The mass was surgically excised. Histological examination showed it to be composed of fusiform cells of variable cellularity with a central hyalinization zone. Immunohistochemical staining was positive for CD34 and negative for smooth muscle actin, S100 protein, desmin, and h-caldesmon. On 1-year follow-up, there was no recurrence of the mass. This case presented some diagnostic difficulty because of the wide range of possible diagnoses for a pediatric scalp mass; however, the distinct immunohistochemical profile helped to eliminate other more frequent fibrous tumors of the scalp. The behavior of scalp SFTs is usually benign, but their course can be unpredictable, and recognition of these lesions is essential.

Stroke in Saudi Arabia.

In this review, the authors summarize 10 years of work on stroke in Saudi Arabia that has led to more than 25 publications. The incidence and prevalence of strokes were low when compared to those reported from Western countries, but this was mainly due to the younger age of the population. The overall distribution of stroke types was not different from that reported in other communities, with the exception of the low incidence of subarachnoid hemorrhage. However, the relatively high frequency of strokes in the young and strokes related to small artery disease, i.e. lacunar infarcts and cerebral hemorrhage, and the high prevalence of diabetes mellitus as a risk factor were quite distinctive. On the other hand, extracranial large artery disease was quite rare. Undetected and untreated hypertension and a low frequency of cigarette smoking in the stroke-prone age group could be one explanation of these findings.

Carpal Tunnel Sydrome: A prospective clinical study of one hundred cases.

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