A clinical study of non-parkinsonian tremor in Moroccan patients

Objective

To report the characteristics of the most frequent tremors in a population of Moroccan patients.

Background

Tremor is the most common movement disorder. It implies a wide variety of disorders with Parkinson's disease and essential tremor being the most frequent.

Methods

A retrospective study of 148 patients with tremor referred to our movement disorders outpatient clinic was performed. Clinical features and treatment regimens were analyzed. Patients with parkinsonian tremor were excluded.

Results

We included 62 patients with non-parkinsonian tremor. The etiologies were as follows: essential tremor (54.8%), dystonic tremor (19.4%), tremor associated with dystonia (14.5%), enhanced physiological tremor (3.2%), cerebellar tremor (3.2%), psychogenic tremor (3.2%) and Holmes’ tremor (1.6%). The characteristics of essential tremor patients were analyzed. Female patients accounted for 67.6% of patients. Mean age at the onset of tremor was 52.2 ± 16.4 years. Family history of tremor was reported in 17.6% of cases. Tremor affected the arms (94.1%), head (52.9%), voice (35.3%) and legs (8.8%). Tremor was bilateral in 87.5% but was asymmetrical in 50% of patients. Patients had postural tremor (76.5%), kinetic tremor (79.4%) and rest tremor (associated in 11.8%). Treatment relied on propranolol (88.3%), primidone (14.7%), gabapentin (14.7%), clonazepam (14.7%), alprazolam (11.8%), topiramate (5.9%) and, in one patient, radiosurgery.

Conclusions

Essential tremor was the predominant diagnosis, confirming its high prevalence. There was a predominance of female patients and a peak of age at onset in the fifth and sixth decades. Asymmetry of the disease was noted in half of patients.     

Percutaneous mitral valvuloplasty in the pregnant woman Moroccan experience

Pregnancy is one of the most important factors known to destabilize valvular heart disease. In particular, pregnancy is a major cause of aggravation in the clinical course of women with mitral stenosis (MS). Surgical treatment of MS during pregnancy may be hazardous for both mother and foetus. In this context, percutaneous mitral valvuloplasty using the Inoue balloon may constitute a particularly attractive alternative to surgery. We report the results of percutaneous mitral valvuloplasty achieved in a series of 11 pregnant women.     

Rôle du Pédodontiste dans le diagnosticde l’infection par le virus d’immunodéficience humaine (VIH) chez l’enfant

Human immunodeficiency virus (HIV) infection is a disease described as having significant and frequent oral manifestations with an early onset character. Which could be a very interesting diagnostic clue. The odonto-stomatologist is therefore in the front line to detect the immunodeficiency caused by this pathology, even though the patient may be unaware of his condition or the aggravation of it, as was the case with our patient.     

Phenotypical features of a Moroccan family with autosomal recessive Charcot-Marie-Tooth disease associated with the S194X mutation in the GDAP1 gene

BACKGROUND: The first locus for demyelinating autosomal recessive Charcot-Marie-Tooth (ARCMT) disease was identified in 8q13, where mutations in GDAP1 have been found. Mutations in the same gene have been detected in families with axonal ARCMT disease. OBJECTIVE: To determine the clinical, electrophysiologic, and morphologic characteristics of a consanguineous Moroccan family with ARCMT disease associated with the S194X mutation in the GDAP1 gene. METHODS: Four patients from a consanguineous Moroccan family were examined clinically and electrophysiologically. In one patient, a morphometric and ultrastructural study of a peroneal nerve biopsy sample was performed. Mutation in the coding region of the GDAP1 gene was identified by direct sequencing. RESULTS: Neuropathy was evident early in childhood, walking was delayed in one patient, and onset of symptoms occurred before 18 months in the others. The phenotype was severe: foot deformities and disabilities involving the hands and feet developed toward the end of the first decade, followed by involvement of proximal muscles in the lower limbs, leading to loss of autonomy. Electrophysiologic findings were consistent with an axonal form of CMT disease: motor nerve conduction velocities, recordable in one patient only, were greater than 40 m/sec. Sensory nerve action potentials were either abolished or substantially reduced in amplitude. The morphologic data supported the diagnosis of axonal neuropathy, showing a marked reduction in myelinated fibers and signs of axonal regeneration, including frequent pseudo-onion bulb formations. The 4 patients in this family were homozygous for the S194X mutation in the GDAP1 gene. CONCLUSION: Electrophysiologic and pathological findings support the hypothesis of an axonal disorder in this ARCMT family with the S194X mutation in the GDAP1 gene.     

Late discovery of Lafora disease: a family study

Lafora disease is a progressive myoclonic epilepsy, Clinically defined by the association of myoclonic, epileptic fits and dementia. We report a case with an atypical Lafora disease, marked by delayed onset at 25 years of age, prolonged course, associated with secondary cognitive impairment and myoclonic features.     

Spontaneous perirenal hematomas. Report of 3 cases]

Spontaneous retroperitoneal hemorrhage is an uncommon affection, the diagnosis was recognized by sonography and CT scan, but the etiology remains unknown and exploration for diagnosis may become necessary. In the absence of an apparent etiology, patients with spontaneous renal bleeding should undergo radical nephrectomy, because of the extremely high incidence of small undetectable occult tumors. Three further cases were reported by the authors, who made a review of the literature.