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To detect the variations of esophageal peristalsis in amyotrophic lateral sclerosis (ALS) patients with predominantly bulbar or predominantly pseudobulbar clinical presentation by using esophageal manometry (EM). Fifteen ALS patients with pseudobulbar clinical presentation (PBP) and 13 patients with bulbar presentation (BP), fulfilling WFN Criteria, were studied. EM was performed in all subjects using a flexible catheter with solid-state transducers. Swallowing was initiated with 5 to 10 mL of water (wet swallows) and saliva (dry swallows) and repeated at 30 s intervals. The manometric parameters were measured automatically and visualized by the computer system. The tracings were analyzed using Synectics software. In PBP patients, an increase of resting pressure value in the upper esophageal sphincter (UES) &gt, 45 mmHg, a wave-like course of resting pressure, and toothed peristaltic waves were observed. In BP patients, a low amplitude of peristaltic waves &lt, 30 mmHg (mean: 17 &plusmn, 5) was recorded, without signs of esophageal motility disturbance at onset or during progression. EM procedure allows objectively distinguishing dysphagia in ALS patients due to bulbar syndrome from the dysphagia due to pseudobulbar syndrome. It is important to identify PBP patients because of their high risk of aspiration.

Rehabilitation treatment in CADASIL disease. A case report Abstract CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a dominant autosomal hereditary angiopathy produced by the muta- tion of NOCTH 3 gene in chromosome number 19. The symptoms of the disease include migraine with aurea, psychiatric problems, ischemic episodes and cognitive deficits. The typical course of CADASIL is slowly progressive. The diagnosis must be genetically confirmed through the detection of its characteristic mutations and/or some cutaneous pathological changes. We present the case of a 63-year-old man who suffered from episodic neurological deficits, left hemiparesis, pseudobulbar syndrome, and progressive sequelae. In February 2004, the dia- gnosis of CADASIL was reached, due to mutation G428A (Gys171Tyr) in exon number 4 of NOTCH

Progressive supranuclear palsy related to neurodegenerative diseases of the brain. In the pathological process involved, and stem structure. The disease manifests a combination of progressive akinetic-rigid syndrome and early development of postural instability leading to frequent falling. Observed supranuclear ophthalmoplegia, pseudobulbar syndrome and dementia of frontal. Arises at the age of 50—60, rapidly progressive, and untreatable protivoparkinsonicheskimi drugs. An clinical case of a patient 59 years old, with these magnetic resonance imaging of the brain.

Different forms of motor neuron disease occurring in association with HIV infection have been described. We present two patients with pseudobulbar syndrome and HIV infection, with no clinical or electromyographic signs of lower motor neuron loss. In patient 1, on follow-up, focal seizures led to additional investigations that identified unsuspected HIV infection and progressive multifocal leucoencephalopathy (PML). In patient 2, all investigations excluded an active HIV infection or central nervous system involvement, and the disease progression made primary lateral sclerosis (PLS) with pseudobulbar onset the most likely diagnosis. ALS-like syndrome can occur in association with HIV infection; however, the causal relationship remains uncertain. Patient 1 shows that PML is a possible cause for pseudobulbar syndrome, and our second patient demonstrates that ALS may also occur by chance in patients with HIV infection.

Objective To describe an 8 year old girl, with a new autosomal recessive syndrome presenting with severe dystonia and on MRI a unique pattern of infolded polymicrogyria. Methods An 8 year old girl is the product of consanguineous Arab-Moslem parents. Prenatal ultrasound demonstrated bilateral clubfeet. She achieved walking at the age of 5.5 years but has no speech. On examination she demonstrates:generalized dystonia, hypotonia with muscle wasting, clubfeet, and a a pseudobulbar syndrome with no expressive speech but good comprehension. Results MRI shows a unique pattern of bilateral infolded extensive perisylvian polymicrogyria. The findings are more prominent on the right side and do not resemble the classical patterns of polymicrogyria. Conclusion To date there is no description of this unique pattern of imaging of perisylvian polymicrogyria. The inheritance of classical bilateral perislvian polymicrogyria is mostly autosomal dominant and this syndrome is assumed to be autosomal recessive due to parental consanguinity. The severe movement disorder in our case has not been mentioned in other perisylvian syndromes.

On rapporte un cas unique de syndrome operculaire du a un abces nocardial de l'hemisphere droit d'un gaucher qui a recupere apres traitement de l'abces

A three generation family is described in which four members were found to have congenital suprabulbar paresis (Worster-Drought syndrome). It is suggested that some cases of congenital suprabulbar paresis are inherited as an autosomal dominant trait with variable expression and penetrance.

(1987). Excessive Yawning Progressive Supranuclear Palsy. International Journal of Neuroscience: Vol. 34, No. 1-2, pp. 123-124.