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cerebral locking syndrome

Yoshinori Kobayashi, Yoshiyuki Hanaoka, Tomoyuki Akiayma, Iori Ohmori, Mamoru Ouchida, Toshiyuki Yamamoto, Makio Oka, Harumi Yoshinaga, Katsuhiro Kobayashi
We report a female patient with Dravet syndrome (DS) with erratic segmental myoclonus, the origin of which was first identified in the cerebral cortex by the detection of myoclonus-associated cortical discharges. The discharges were disclosed through jerk-locked back-averaging of electroencephalogram (EEG) data using the muscle activity of myoclonus as triggers. The detected spikes on the contralateral parieto-central region preceded myoclonic muscle activity in the forearms by 28-46ms. The patient was six months old at the time of examination, and was developing normally before seizure onset at two months of age...
August 11, 2016: Brain & Development
Andrea Soddu, Francisco Gómez, Lizette Heine, Carol Di Perri, Mohamed Ali Bahri, Henning U Voss, Marie-Aurélie Bruno, Audrey Vanhaudenhuyse, Christophe Phillips, Athena Demertzi, Camille Chatelle, Jessica Schrouff, Aurore Thibaut, Vanessa Charland-Verville, Quentin Noirhomme, Eric Salmon, Jean-Flory Luaba Tshibanda, Nicholas D Schiff, Steven Laureys
INTRODUCTION: The mildly invasive 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) is a well-established imaging technique to measure 'resting state' cerebral metabolism. This technique made it possible to assess changes in metabolic activity in clinical applications, such as the study of severe brain injury and disorders of consciousness. OBJECTIVE: We assessed the possibility of creating functional MRI activity maps, which could estimate the relative levels of activity in FDG-PET cerebral metabolic maps...
January 2016: Brain and Behavior
Eileen Broomall, Mary E McBride, Barbara J Deal, Laurence Ducharme-Crevier, Alexandra Shaw, Mjaye Mazwi, Carl L Backer, Michael C Mongé, John Costello, Bradley S Marino, Andrew DeFreitas, Mark S Wainwright
BACKGROUND: Palliative procedures performed before the Fontan procedure may require ligation of the subclavian arteries, thereby affecting flow to the vertebral arteries. In adults with functionally univentricular heart disease, the implications of altered brainstem vascular anatomy for perioperative management of failing Fontan circulation are not known. METHODS: We identified abnormal posterior circulation anatomy in an adult patient with failing Fontan circulation who experienced a brainstem stroke after Fontan conversion...
June 2016: Annals of Thoracic Surgery
Lasanthi Aryasinghe, Yasmin Kazim, Hamza F Obeid, Husnain Hashim
BACKGROUND: Locked-in syndrome, although a notoriously famous clinical entity, the rarity of the condition coupled with the variability of clinical features on acute presentation represents a potential diagnostic pitfall for the emergency physician. CASE: A previously healthy 25-year-old female was brought to our Emergency Department after being found unresponsive. On examination, she was conscious and alert with a Glasgow Coma Score of 9; on neurological examination, the patient was quadriplegic and unable to speak but was able to move her eyes and blink...
December 2016: International Journal of Emergency Medicine
Giuseppe d'Orsi, Luigi M Specchio
This study aimed to elucidate the natural history of senile myoclonic epilepsy, a type of myoclonic epilepsy associated with Alzheimer's disease in adult Down syndrome patients. Twelve Down syndrome patients over the age of 40 years with myoclonic epilepsy and Alzheimer's disease underwent clinical, neuropsychological, neurophysiological, and neuroradiological study. The kariotypes, APOE polymorphisms, all exons in the PSEN1 and PSEN2 genes, and exons 16 and 17 in the APP gene were determined for all patients...
August 2014: Journal of Neurology
Johan Stender, Olivia Gosseries, Marie-Aurélie Bruno, Vanessa Charland-Verville, Audrey Vanhaudenhuyse, Athena Demertzi, Camille Chatelle, Marie Thonnard, Aurore Thibaut, Lizette Heine, Andrea Soddu, Mélanie Boly, Caroline Schnakers, Albert Gjedde, Steven Laureys
BACKGROUND: Bedside clinical examinations can have high rates of misdiagnosis of unresponsive wakefulness syndrome (vegetative state) or minimally conscious state. The diagnostic and prognostic usefulness of neuroimaging-based approaches has not been established in a clinical setting. We did a validation study of two neuroimaging-based diagnostic methods: PET imaging and functional MRI (fMRI). METHODS: For this clinical validation study, we included patients referred to the University Hospital of Liège, Belgium, between January, 2008, and June, 2012, who were diagnosed by our unit with unresponsive wakefulness syndrome, locked-in syndrome, or minimally conscious state with traumatic or non-traumatic causes...
August 9, 2014: Lancet
K Brukamp
Motor-independent communication is a novel diagnostic and therapeutic method that is currently in development in order to enable communication with severely physically challenged patients. Some patients with locked-in syndromes or with chronic disorders of consciousness are capable of modulating their brain activities to such a degree that the latter can be analyzed regarding communicative intentions with neuroscientific technologies, such as functional magnetic resonance imaging. Further scientific development and an increasing clinical use of motor-independent communication will aid in meeting essential quality standards for this method...
October 2013: Der Nervenarzt
Troels W Kjaer, Helge B Sørensen
Brain-computer interfaces (BCI) register changes in brain activity and utilize this to control computers. The most widely used method is based on registration of electrical signals from the cerebral cortex using extracranially placed electrodes also called electroencephalography (EEG). The features extracted from the EEG may, besides controlling the computer, also be fed back to the patient for instance as visual input. This facilitates a learning process. BCI allow us to utilize brain activity in the rehabilitation of patients after stroke...
2013: Frontiers of Neurology and Neuroscience
Boris Kotchoubey, Martin Lotze
The locked-in syndrome (LiS) is typically characterized by a paralysis of almost all body muscles combined with intact cognitive functions. In practice, there are often additional brain damages besides the one directly causing LiS. These damages can lead to cognitive impairment, which substantially complicates the diagnosis of LiS. At the level of behavior, therefore, the disease can be confused with akinetic mutism, vegetative state (syn. unresponsive wakefulness state) and some other conditions. Using instrumental methods in addition to behavioral diagnostics helps to avoid diagnostic errors and to improve prognosis of rehabilitation of such patients...
2013: Restorative Neurology and Neuroscience
P Hantson, X Wittebole, P Rombaux, G Cosnard
BACKGROUND: A 32-year-old woman developed altered consciousness two days after initial symptoms of acute otitis media, with purulent discharge from the right ear. She was quadriplegic, with spontaneous eye opening, mild neck stiffness, and lacking vestibular-ocular reflexes. METHODOLOGY: Upon admission, the patient was subjected to brain computed tomography (CT), magnetic resonance imaging (MRI), and lumbar puncture. RESULTS: CT was consistent with pansinusitis, right middle ear otitis, mastoiditis, and sphenoiditis...
2012: B-ENT
Indranil Sen-Gupta, David A Daiga, Mark J Alberts
No abstract text is available yet for this article.
June 12, 2012: Neurology
Aurore Thibaut, Marie-Aurélie Bruno, Camille Chatelle, Olivia Gosseries, Audrey Vanhaudenhuyse, Athena Demertzi, Caroline Schnakers, Marie Thonnard, Vanessa Charland-Verville, Claire Bernard, Mohammed Bahri, Christophe Phillips, Mélanie Boly, Roland Hustinx, Steven Laureys
OBJECTIVE: An extrinsic cerebral network (encompassing lateral frontoparietal cortices) related to external/sensory awareness and an intrinsic midline network related to internal/self-awareness have been identified recently. This study measured brain metabolism in both networks in patients with severe brain damage. DESIGN: Prospective [18F]-fluorodeoxyglucose-positron emission tomography and Coma Recovery Scale-Revised assessments in a university hospital setting...
May 2012: Journal of Rehabilitation Medicine
T Lock, J McCulloch
The [(14)C]2-deoxyglucose autoradiographic technique measures local rates of glucose utilization simul taneously in all functional and anatomical subunits of the CNS and provides a measure of the functional consequences in the CNS of any manipulation. Glucose utilization was measured in 24 regions of the CNS in conscious rats one day after the end of a 7-day course of electroconvulsive shock (chronic ECS). Chronic ECS had minimal effects on local glucose utilization in most brain areas (including extrapyramidal, cortical, hippocampal and other limbic structures) with only the nucleus accumbens displaying a significant alteration (reduced by 25% from sham-treated animals)...
January 1991: Journal of Psychopharmacology
Yasunori Fujimoto, Yu-ichiro Ohnishi, Akatsuki Wakayama, Toshiki Yoshimine
Locked-in syndrome (LIS) usually occurs as a result of pontine lesions and has been classified into various categories on the basis of neurologic conditions, of which transient total mesencephalic LIS is extremely rare. A 53-year-old man presented with bilateral ptosis followed by a total locked-in state. In the clinical course, the patient successfully recovered with only left slight hemiparesis and skew deviation remaining. Magnetic resonance imaging revealed multiple ischemic lesions caused by thrombosis at the top of basilar artery, including the bilateral cerebral peduncles, tegmentum of the midbrain, and the right cerebellar hemisphere...
November 2012: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
G Lacroix, D Couret, X Combaz, B Prunet, N Girard, N Bruder
BACKGROUND: Cerebral vasospasm is the main cause of neurological mortality and morbidity following subarachnoid hemorrhage. Basilar artery vasospasm (BAVS) is associated with a high morbidity and may have multiple clinical presentations. METHODS: We report the case of a 43 years-old man with BAVS presenting as a reversible locked-in syndrome (LIS) after stopping sedation. RESULTS: The symptoms were successfully managed by intra-arterial infusion of vasodilators and balloon angioplasty...
February 2012: Neurocritical Care
W-D Heiss
Advances in resuscitation and critical care management have resulted in the survival of many patients despite severe brain damage. These patients may remain in coma or in vegetative state. The probability of recovery of conscious function is dependent on the extent of structural brain damage, which is difficult to assess by clinical, laboratory or functional tests. Positron emission tomography (PET) of 18F-fluordeoxyglucose (FDG) can be used to investigate metabolic and functional impairment of the brain. In acute vegetative state (AVS, duration <1 month), overall glucose utilization was significantly reduced in comparison with age-matched controls...
February 2012: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Sorin Aldea, Pierre Guedin, Luca Roccatagliata, Anne Boulin, Stéphanie Auliac, Michel Dupuy, Charles Cerf, Stéphan Gaillard, Georges Rodesch
Intracavernous carotid artery aneurysms (ICCAs) are rarely associated with life-threatening complications. We describe a 55-year-old woman who, after the rupture of an intracavernous carotid artery aneurysm, presented with a contralateral cavernous sinus syndrome and severe posterior fossa and spinal cord symptoms. Following parent artery occlusion, thrombosis of the posterior fossa and spinal cord veins caused a progressive worsening of the neurological status to a "locked-in" state. The patient fully recovered with anticoagulation therapy...
June 2011: Acta Neurochirurgica
Mano Shanmuganathan, Charles P Warlow, Rustam Al-Shahi Salman, Peter Keston, Robin J Sellar, Ioannis P Fouyas
We present a case of a 48-year-old man who was initially thought to have had a brainstem stroke and was clinically 'locked-in'. Upon investigation, a petrous apex dural atriovenous fistula was identified causing profound brainstem venous hypertension. Surgical clipping lead to complete neurological recovery.
February 2011: British Journal of Neurosurgery
François Cotton, Sophie Ciancia, Laurence Tell, Laurence Lachaise, Marc Braun, Gilles Rode
A 52-year-old woman has been under observation for a complete locked-in syndrome of vascular origin, since 1984. Her cognitive functions today are still normal. When first diagnosed, a CT-scan was made and 23 years later performed, a cerebral MRI was performed. A focal, bilateral and symmetric atrophy of the dorsomedial prefrontal gyri was clearly shown, contrasting with the non-atrophy of the precentral gyri (motor area), others prefrontal areas, frontopolar gyri and temporal cortices. Degeneration of the corticopontine projection, the first step in the corticopontocerebellar circuit, could explain this selective atrophy...
May 2011: Journal of Neuroradiology. Journal de Neuroradiologie
Spencer Kellis, Kai Miller, Kyle Thomson, Richard Brown, Paul House, Bradley Greger
Pathological conditions such as amyotrophic lateral sclerosis or damage to the brainstem can leave patients severely paralyzed but fully aware, in a condition known as 'locked-in syndrome'. Communication in this state is often reduced to selecting individual letters or words by arduous residual movements. More intuitive and rapid communication may be restored by directly interfacing with language areas of the cerebral cortex. We used a grid of closely spaced, nonpenetrating micro-electrodes to record local field potentials (LFPs) from the surface of face motor cortex and Wernicke's area...
October 2010: Journal of Neural Engineering
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