Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.

Pallido-luysio-nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62-year-old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2-weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO-SPECT and FDG-PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.

[Paroxysmal neurological manifestations disclosing panic attacks]. / Manifestations neurologiques paroxystiques révélatrices d'attaques de panique.

Thirty-seven patients presented with paroxysmal neurological manifestations attributed to anxiety attacks. The manifestations included loss of consciousness, focal sensorimotor deficits, diffuse dysesthaesiae, visual disorders and tremor. They lasted 10 to 45 minutes and occurred once per day to once per week. Organic pathology was dismissed on the basis of normal examinations and atypical course. In all patients questioning revealed symptoms that were those of acute anxiety. The fact that these attacks took place in suggestive (circumstances e.g. in crowds and car driving), and that they could be induced by challenge tests hyperpnoea, infusion of lactate) suggested that these disorders were consecutive to panic attacks.