Wednesday, January 28, 2009

Styloid syncope

Neuroimages p 1649 Neurology71 2008
Patient had craniocervical pain and 30 years of syncope preceded by neck flexion and brief vertigo. 3D CT showed hypertrophied styloid process.

Eagle syndrome is characterized by a long SP with craniocervical or throat pain. Elongate SP occurs in 4 % of population. Syncope is due to compression of carotid artery. It should be considered in young patients with chronic throat pain and syncope provoked by neck flexion. It is diagnosed with 3d Ct and cured with styloidectomy.

foreign language ictal speech automatisms

Neurology 2008; 71: 1579-1585
Most cases reflect right TLE. However, FLISA occur with spread to temporal neocortex of frontal operculum. Authors suggest a male preponderance although this blogger's only personal observation is a woman.

Tuesday, January 20, 2009

Brain calcinosis syndrome

ref Baba et al. Heredofamilial brain calcinosis syndrome Mayo Clin Proc 2005;80:641-651
(a little dry for this blog)

List of causes:

80+% clinically benign
sporadic or familial I With abnormal calcium or magnesium metabolism
Hypoparathyroidism (idiopathic, postsurgical, external radiation, hypomagnesemia)
Pseudohypoparathyroidism (type II, idiopathic)

II Without Ca/Mg abnormalities
Down's syndrome
Mitochondrial neuromyopathy (Kearns-Sayre syndrome, Pearson s.)
IgG kappa M proteinemia
Revesz syndrome
infectious (toxopl, mumps, EBV, CMV, HIV)
angiomatous malformations of the vein of Galen
Toxic (lead, CO)
Therapy induced ( XRT, anticancer drugs, mineralizing microangiopathy)
Without systemic involvement
Diffuse NFT with calcification
hyperkinetic mutism
idiopathic (Fahr's dis, striatopalidodentate calcinosis, bg calcification)

Genetic syndromes with chromosome identified (see reference for actual gene loci)

Familial isolated hypoparathyroidism
Autoimmune polyglandular syndrome
Pseudohypoparathyroidism types Ia and Ib
Aicardi syndrome (in first year of life, may see calcium in thalamus, cerebellum also)
Dihydropterine reductase syndrome (=PKU type 2 atypical form)-- may be arrested by folic
Cockayne syndrome
Others (see text)

Tuesday, January 13, 2009

localizing signs with seizures

Clinical lateralizing signs are the phenomena which can unequivocally refer to the hemispheric onset of epileptic seizures. They can improve the localization of epileptogenic zone during presurgical evaluation, moreover, their presence can predict a success of surgical treatment. Primary sensory phenomena such as visual aura in one half of the field of vision or unilateral ictal somatosensory sensation always appear on the contralateral to the focus. Periictal unilateral headache, although it is an infrequent symptom, is usually an ipsilateral sign. Primary motor phenomena like epileptic clonic, tonic movements, the version of head ubiquitously appear contralateral to the epileptogenic zone. Very useful lateralization sign is the ictal hand-dystonia which lateralizes to the contralateral hemisphere in nearly 100%. The last clonus of the secondarily generalized tonic-clonic seizure lateralizes to the ipsilateral hemisphere in 85%. The fast component of ictal nystagmus appears in nearly 100% on the contralateral side of the epileptic focus. Vegetative symptoms during seizures arising from temporal lobe such as spitting, nausea, vomiting, urinary urge are typical for seizures originating from non-dominant (right) hemisphere. Ictal pallor and cold shivers are dominant hemispheric lateralization signs. Postictal unilateral nose wiping refers to the ipsilateral hemispheric focus compared to the wiping hand. Ictal or postictal aphasia refers to seizure arising from dominant hemisphere. Intelligable speech during complex partial seizures appears in non-dominant seizures. Automatism with preserved consciousness refers to the seizures of non-dominant temporal lobe.