The locomotive syndrome

This syndrome was first described in 2007 by the Japanese Orthopedic Association in order to define a new concept causing gait disorders in a considerable number of very elderly patients. This is the locomotive that doesn’t pushes the train forward. The neurologist often meets these patients.
The locomotive syndrome is the consequence of pathologies on 3 levels: bone (osteoporosis), articulations and vertebral discs (spondylosis and spondyloarthritis at the cervical and lumbar level, shoulders, hips and knee arthritis), muscles and peripheral nerves. Finally the locomotive syndrome brings together several pathologies that are related to each other. The symptoms are pain, limitation of mobility, difficulties of balance and walking.
The consequence of this syndrome is a decrease of the functional autonomy for the activities of daily living, a decrease in social life and quality of life. There is a 25-item questionnaire (Geriatric Locomotive Function Scale – GLFS-25) that can help to establish the diagnosis. Other measures can be used to better define the functional state such as the handgrip strength, the on one leg time standing, and the 6 m walking time The syndrome is common for elderly patients. While the term « frailty syndrome » is intended in case several organs are typo-functioning (cognition dysfunction is included), the term locomotive syndrome applies to multiple inter related musculoskeletal problems.
Osteoporosis is an important component of the locomotive syndrome and its prevention is fundamental. Finally, to prevent the locomotive syndrome of the geriatric age, the most important thing to do is to let patients moving. Favor mobility by all means. Assessment and treatments are provided by a multidisciplinary team. Thus, do not forget the neurologist.

Pubblicato in Neurorehabilitation | Lascia un commento

Migraine and sleep

We know, as neurologists, that there is an association between migraine and poor sleep. Sleeping poorly (non-restorative sleep, insomnia) increases migraine attacks and worsens comorbidities (depression and anxiety).
It is known that the brain of the patients with migraine is more sensitive to environmental stimuli (only one example: the light) and that their visceral-vegetative system is more active. There is scientific evidence that migraine patients need more sleep than others. Poor sleep decreases the pain tolerance of migraine sufferers, but also of patients with tension headache.
On the other hand, improving sleep can turn a chronic migraine into an episodic one.
Sleeping pills may be helpful initially, but it is not a valid long-term solution for migraine.
Some tips for migraine sufferers to improve sleep:
⁃ Behavioral cognitive therapy targeted at sleep, especially in cases of depression or anxiety
⁃ No coffee after 1 pm
⁃ No alcohol at night
⁃ No exercising in the 5 hours before bedtime
⁃ Shower (not too hot) before bedtime
⁃ No fluids within 2 hours before bedtime
⁃ Interval of at least 4 hours between dinner and bedtime
⁃ No lights in the bedroom during the night
⁃ The alarm clock must not be visible (turn it)
⁃ Switch-off smartphones and tablets
⁃ No television in bed
⁃ The bed is only for sleeping (or for couple intimacy)
⁃ Listen, if necessary, to music that facilitates sleep (reiki, Marconi Union https://www.youtube.com/watch?v=vm_h0VWj2dk…), without words
⁃ Go to bed when you know you could fall asleep in 20 minutes
⁃ To sleep at least 8 hours every day included weekend (to avoid the migraine of the weekend)
⁃ Get up every day at the same time (to avoid the migraine of the weekend)
⁃ Do not nap during the day

Do you have other tips? Please share with us your experiences.

Pubblicato in Headache, Sleep, Uncategorized | Lascia un commento

Misidentification syndromes at the interface of neurology and psychiatry

As neurologists, sometimes we deal with special « delusions ».
Misidentification syndromes are rare neurologic conditions in which the patient attributes a wrong identity (a sort of hypo-identification) to people (Capgras syndrome or doubles’ illusion), to places (reduplicative amnesia), or believes that the physical appearance of a person changed into that of another (Fregoli syndrome), this last condition corresponds to a sort of hyper-identification. A parallelism can be traced between the Capgras syndrome and somatoparaphrenia (a right hemisphere syndrome). The patient with somatoparaphrenia believes that his or her paralyzed left arm belongs to another person.
The patient with the Capgras syndrome believes that his spouse has been replaced by another person or an alien. So, even if her physical aspect is the usual, inside there is an impostor.
Despite all plausible evidence, there is no way to persuade him that he is wrong. This situation well reminds the 1957 American science fiction horror movie « the invasion of the body snatchers », a cornerstone of extraterrestrial invasions in movies. The patient does not understand that no-one believes him on the false identities of people.
The patient with reduplicative amnesia believes that his house, despite a normal aspect, has been replaced by another location.
More types of misidentification can coexist in the same patient.
All these conditions are at the interface between neurology and psychiatry as they can manifest either with neurologic disease (stroke, subdural hematoma, Alzheimer’s disease, Lewy-body disease, drug intoxication, brain trauma, Parkinson’s disease, Fahr’ disease, levodopa-induced psychosis, epilepsy) or psychiatric disease without brain lesions (such as paranoia, schizophrenia, mania, and dissociative disorders).
Capgras syndrome and reduplicative amnesia manifest after frontal parietal, occipito-parietal, and thalamic lesions. The neural mechanisms underlying the dissociation between appearance and identity of the bodies remain speculative. However, it points to the existence of different cognitive and emotional networks for recognition (or memory) of faces and bodi, networks having different neural substrates or hemispheric dominance.
If you think that your spouse has been replaced by an alien or that your house is another place consult us as something could be wrong in specific regions of your brain.

Pubblicato in Cognition and Behavior, Lewy Body Disease, Parkinson Disease, Psychiatry | Lascia un commento

Fibromyalgia and repetitive transcranial magnetic stimulation (rTMS)

We provide in our centre brain neruomodulation with rTMS in order to treat patients with fibromyalgia. Fibromyalgia is a very common chronic painful musculoskeletal condition affecting about 1-3% of the population world wide. Main clinical features are widespread muscular pain and tenderness, commonly associated with fatigue, sleep disturbances, memory and attention deficits and mood disorders (anxiety and depression). Diagnosis is made according to the American College of Rheumatology criteria (Wolfe et al. 2010 and 2016). A rheumatologist should provide the diagnosis.
The aetiology of the disorder is unknown and there is no a definite cure. Pharmacological interventions (anticonvulsants, antidepressants, AINS and opioid-like substances) have limited efficacy and often side effects. Psychotherapies and physiotherapy have partial success.
Several experimental studies on fibromyalgia suggested the key role of chemical, structural and physiological changes of the central nervous system for pain maintenance. An increased neuronal excitability is installed in brain areas processing physical, emotional and cognitive attributes of pain, probably mediated by glutamate activity on the NMDA receptors.
Repetitive transcranial magnetic stimulation (rTMS) is a safe and non-invasive procedure that uses a magnetic field to modulate the activity of cortical brain areas and their networks and to reduce glutamate activity.
Contraindications for TMS are epilepsy or history of seizure, pacemakers and other implantable medical devices, serious head injury, pregnancy. rTMS treatment can be easily performed on outpatients.
Even if there are only weak recommendations provided by meta-analytic studies and further clinical research is needed, several studies demonstrated the effectiveness of rTMS excitatory modulation on the fronto-dorso-lateral cortex over several weeks. There is expected a minimum 30% of pain improvement and a similar significant chance of fatigue reduction and improvement in sleep and quality of life.

REFERENCES

Fitzgibbon BM et al. Evidence for the improvement of fatigue in fibromyalgia: a 4-week left dorsolateral prefrontal cortex repetitive magnetic stimulation randomized-controlled trial. Eur J Pain 2018 (epub ahead of print).

Hou WH et al. The effects of add-on non-invasive brain stimulation in fibromyalgia : a meta-analysis and meta-regression of randomized controlled trial. Rheumatology (Oxford)2016.55(8):1507-17.

Boyer L. et al. rTMS in fibromyalgia: a randomized trial evaluating QoL and its brain metabolic substrate. Neurology 2014.82(14):1231-8.

Lee SJ et al. The effect of repetitive transcranial magnetic stimulation on fibromyalgia: a randomized sham-controlled trial with 1-mo follow-up. Am J Phys Med Rehabil 2012.91(12): 1077-85.

Pubblicato in neuromodulation, rTMS | Lascia un commento

Recherche de médecin spécialiste

Nous désirons developper ultérieurement notre centre de neurologie et nous cherchons (à temps plein ou à temps partiel) un médecin indépendant d’une autre spécialité (possiblement apparentée avec la neurologie ou un psychiatre ou un spécialiste de médecine interne) avec un droit de pratique dans le canton de Vaud. Situé dans un élégant immeuble à l’entrée de Gland notre spacieux cabinet met à disposition une salle de consultation. Le centre dispose de plusieurs salles de consultations, une salle d’examen, une cuisine agencée et une grande réception/salle d’attente. Le centre est accessible aux fauteuils roulants. Le cabinet est en proximité d’arrêt de bus (en face), il se trouve à la sortie de l’autoroute de Gland et dispose de 6 places de parkings. Pour d’autres informations visiter le site www.neurolacote.ch. Vous pouvez nous contacter par e-mail : neuro-la-cote@bluewin.ch ou par téléphone pendant les heures de bureau 022 995. 97. 44.

Pubblicato in Uncategorized | Lascia un commento

The Balint syndrome (a chaotic visual world)

The Balint syndrome is a rare neurological condition consisting of a triad of visuomotor deficits : simultanagnosia, oculomotor apraxia and optic apraxia.
Simultanagnosia is the inability to perceive objects when they are simultaneously presented (seeing the tree but not the forest). Patients with simultanagnosia see the world in pieces (piecemeal vision) and not in its integrity.
Oculomotor apraxia consists of the difficulty to direct voluntarily the gaze to an object. Optic apraxia is the condition of not being able to grasp objects by hands with the visual guidance.
Finally, the patients with the Balint syndrome act as they were blind. Their vision is a patchwork, they cannot perceive more than an object at a time or understanding complex visual scenes. They cannot move the eyes where they want and they cannot grasp the objects that they see.
The syndrome (often not recognized) is the result of bilateral ischemic strokes involving the parieto-occipital regions, interrupting the connections between primary visual areas of the occipital lobe and secondary visual areas of the parietal lobe.
Rehabilitation of patients with the Balint syndrome is extremely difficult and would be based on attempts of sensory modalities crossed interaction (visual, tactile, auditory and kinesthetic) or on virtual reality applications.
We cannot ever imagine how such a visual world this would be (seeing without perceiving).
In our cabinet we can perform complex visual assessments to diagnose the Balint Syndrome or other supramodal visual disorders.

Pubblicato in Apraxia, Cerebrovascular diseases, Cognition and Behavior, Neurorehabilitation, Stroke, visual disorders | Lascia un commento

Occipital neuralgia

The occipital neuralgia (formerly called Arnold’s neuralgia) is a primary headache, which must be distinguished from the migraine.
The main symptom is an electric, burning and invalidating pain (generally different from the migraine), of which the origin is just below the occipital protuberance and which radiates anteriorly to the vertex of the skull. The patients describes the location of the pain at a superficial level on the scalp. The pain increases by combing the hairs or pressing the head on the pillow.
The great and\or the lesser occipital nerves are involved.
Clinically, the pain and tingling are reproduced by the palpation of nerves next to the occipital bone (Tinel’s sign).
Pain mechanisms are not completely understood. Nerves are probably compressed somewhere by the oblique and the trapeze muscles.
Indeed, the diagnosis seems confirmed by the disappearance of the pain by an anesthetic nerve block. In rare cases the nerve irritation is secondary to vascular malformations, infectious or inflammatory diseases (eg myelitis and multiple sclerosis), vasculitis and arteritis (such as Horton’s disease in the elderly)  or compression of the C2 root, which is at the origin of the occipital nerve, at the vertebral level. A herpetic infection of the C2 root may mimic occipital neuralgia (but in this case we must search for the herpetic vesicular lesions on the scalp). Sometimes neuralgia occurs after cervical spinal surgery.
In suspicion of infiltrative or structural lesions, it is advisable to perform a brain / cervical MRI exam.
Pharmacological treatments (NSAIDs, pregabalin, muscle relaxants) can be initiated. Nevertheless, as symptoms are often excruciating, we perform already at the beginning, the anesthetic block of the nerve by Lidocaine (without corticosteroids) or a local treatment of mesotherapy (Lidocaine+Amytriptyline).
The latest resources for chronic pain which is refractory to standard therapies are pulsed radiofrequency, implantation of an occipital nerve stimulator, and botulinum toxin infiltrations.

Pubblicato in Headache, Pain syndromes | Lascia un commento

Minor Hemisphere Major Syndromes

Dr Carota recently published an extensive article entitled “Minor hemisphere major syndromes” on a prestigious international journal of neurology. You can take a look to understand how the functional differences of the two brain hemispheres result in different syndromes in case of stroke or other neurological diseases.

Abstract

A right “minor hemisphere” does not exist as the right hemisphere is dominant for awareness (nosognosia), spatial attention, emotional regulation, facial and voice expressions, visual recognition, and topographical orientation. Without the right hemisphere, the world would be flat, deprived of general and spatial attentions, pointing preferentially to the right side of the space, lacking visual experiences and emotions, exhibiting diminished awareness of the self and environment. Clinical-related syndromes of the right hemisphere are unilateral spatial neglect, object and face visual agnosia, the anosognosia for hemiparesis and/or hemianopia, misidentification syndromes, mania, and other obsessions for the food and the body. Another key function of the right hemisphere is the modulation of the emotional processes of the linguistic communication (as prosody and facial expressions), and the tuning of some holistic aspects of language as the understanding of the abstract and figurative characters. The great mysteries of the right brain hemisphere concern the origin of the emotional nature of the human being, the way by which cognition interacts with perception and finally the human consciousness. Multidisciplinary researches in the domains of neurology, cognitive psychology, neuropsychiatry, functional neuroimaging, and neurophysiology will reveal in the future some of these mysteries

 

Pubblicato in Cognition and Behavior, Stroke | Lascia un commento

MINI-COG: to do absolutely

The MINI-COG (Borson and al. 2000) is an excellent screening test for dementia and Alzheimer’s disease. I always suggest to general doctors or to relatives to perform the test to the person with memory difficulties.
The test requires only three minutes of time. It is a very easy and excellent screening test.
It is necessary to cite three words (BANANA-SUNRISE-CHAIR) and let him repeat, asking to memorize the words for the future.
Afterwords, it is necessary to give to the subject the instructions to draw ta clock. He has to write the numbers and put the hands at 11:10. These consignes can be repeated but no additional instruction must be given. There is no time limit.
Afterwords, we ask the subject to recall the three words (1 point for every correct word, the order is not important).
The clock is considered normal or abnormal; normal if all the numbers are present with good sequence and position with the hands indicating the desired hour.
The test is positive (indicating a probability of 90 % of dementia or Alzheimer disease) if the clock is abnormal and there is a score of 0, 1 or 2 for the remembered words. The test is negative with a score of 3 for the words, or 1 or 2 for words together with an abnormal clock.
The figure shows the clock of a patient with an Alzheimer’s disease at a late stage.
The sensibility of the MINI-COG is high. If the test is positive there is approximately 80-90 % of probability that the patient has dementia or Alzheimer’’s disease, while the false negatives are below 10).
If the test is positive you should not hesitate to contact us, because, in these cases, it is necessary to make a diagnosis as soon and as accurate as possible.

Pubblicato in Alzheimer, Cognition and Behavior, Memory | 1 commento

Mesotherapy onboarding


For the treatment of the acute and chronic painful syndromes, besides the usual oral drugs, especially in case of non response, it is often necessary to make recourse to other treatments. Among them, the mesotherapy has an important place. The mesotherapy is now part of the inter-university course of the French medical schools.

In our center we provide Mesotherapy mainly for headache and cervical pain.

The mesotherapy consists in very superficial and not painful local injections, through the skin, of the lowest quantities of the classic drugs.  The basic principle of the mesotherapy is to inject small quantities of drugs where the pain it is.
These injections can be intra epidermic, intradermal superficial or deep, generally, between 2 and 4 mm under the surface of the skin. We use very fine needles (Ø 0.4mm) and single-use material.

The injectable products are the ones of the general medicine, but in very small quantity and namely: myorelaxants, anti-edema, anti-inflammatory drugs vasodilators, multivitamins, etc. Once injected, the drug remains in the site of injection to be released locally and slowly. There are almost never side effects. Doses are very low and there are no unfavorable interactions with the the oral traitements. The intra-epidermic injections are feasible also in case of patients treated by oral anticoagulants, because there is any hemorrhagic risk. The mesotherapy turns out to be particularly interesting for the older individuals, in particular those who already received  many standard oral drugs and want and/or can try another route of administration. There is no risk of HIV or viral hepatitis. All the precautions of antisepsis are taken.

The mesotherapy can lead rather quickly to the disappearance of a pain or its cure but only when the diagnosis is correctly provided. We have to know exactly what we treat. If radiological investigations are necessary to obtain a diagnosis, they must be done before mesotherapy. In the case of pain due to discal hernia, mesotherapy can be done even before recurring to deep infiltrations with CT-guide.

Generally, we provide 2-3 treatments (with a delay of 8-10 days among them) and, in the chronic cases, if there is a positive effect, the treatment is repeated every 3-4 months.
The administration of corticoids is forbidden with mesotherapy. The mesotherapy is a medical act and should be done by doctors. You can find the list of the doctors exercising the mesotherapy  Switzerland on the site of the Swiss society of Mesotherapy (www.mesotherapie.ch).

The Dr A. CAROTA is a member of the Swiss society of mesotherapy and uses this treatment for headache and cervical pain, also for the migraine (preventive treatment), arthrosis pain or the neurogenic pain (neuralgias, focal neuropathies, radiculopathies) or post-traumatic pain of the spine and the limbs. The mesotherapy has also a place also for the treatment of the postherpetic neuralgia or its prevention.

Pubblicato in Pain syndromes, Uncategorized | 1 commento

Vitamine D and Multiple Sclerosis (MS)

Vitamin D is an essential nutrient for bones and metabolism of calcium. However, its deficiency has been related to many disorders and to higher risks of mortality. Vitamine D regulates the activity of 2000 genes and its production is enhanced by the exposition to the sun.
Vitamin D deficiency or insufficiency has been defined as a serum level of 25(OH)D <50 nmol/L or 52.5–72.5 nmol/L, respectively. Deficiency is common across all age groups, especially in case of limited exposure to the sun. The daily intake should be of 400-800IU/day and commonly prescribed regimen consists of 2000-3000IU/day (in order to achieve optimal serum levels even in obese patients). Vitamin D toxicity is very rare, with none seen at doses up to 20000 IU/day.
There is enough evidence for the protective role of vitamine D for patients with autoimmune diseases including MS. The vitamin D interacts with the major histocompatibility complex of the immune cells and contributes to the production of interleukins and immunoglobulins.
Actually, a higher level of sun exposure and vitamin D intake as well as higher serum levels of 25(OH)D, are known to associate with a lower risk of MS. Data from some clinical studies indicate that Vitamine D (alone or in conjunction with interferon) improves the clinical course of MS, such as lowering the risk of relapses and reducing MRI brain lesions. Although this data should be further confirmed in specifically designed randomized clinical study we already propose vit D supplementation as an add-on treatment to standard immunomodulatory drugs to patients with multiple sclerosis. As vitamine D supplements, we believe that, finally, there is nothing to lose for patients with MS.

Pubblicato in Multiple Sclerosis | Lascia un commento

Repetitive Transcranial Magnetic Stimulation (rTMS)

Dans notre centre de Neurologie à Gland, nous avons maintenant la possibilité d’effectuer des traitements de stimulation magnétique transcrânienne répétitive (rTMS).

Nous sommes les seuls neurologues installés, de la Suisse romande à proposer ce traitement ambulatoire.

Il s’agit d’une méthode non invasive de stimulation magnétique directe du cortex cérébral (environ 2000-3000 stimulations par séance). En fonction de la fréquence de stimulation, il est possible d’obtenir un effet d’excitation ou d’inhibition sur le cerveau. Par exemple si l’on stimule la région motrice de la main de la région prérolandique, la main controlatérale à la stimulation bouge. L’efficacité thérapeutique de la rTMS est désormais reconnue par un grand nombre d’études scientifiques dans le cas de certaines maladies neurologiques et psychiatriques. Ces études ont permis de définir des protocoles paramétrés de rTMS (nombre de stimuli, intensité et fréquence de stimulation) et de définir les régions cérébrales cibles. Les régions de stimulation sont localisées sur le crâne sur la base d’algorithmes standard.

La rTMS n’a pratiquement pas ou presque pas d’effets secondaires. Le traitement est ambulatoire et il n’y a aucune sédation ou anesthésie qui est nécessaire.     Ce traitement est néanmoins contre-indiqué chez les personnes porteuses d’implants cochléaires, pace makers et défibrillateurs cardiaques, ainsi que les patients épileptiques.
Les effets positifs du traitement apparaissent en général après 2-3 semaines de traitement.

La rTMS est indiqué comme thérapie pour les douleurs neurogènes d’origine centrale, la migraine chronique et autres conditions douloureuses par exemple la fibromyalgie

L’héminegligence spatiale et l’aphasie après accident vasculaire pourraient aussi bénéficier de la rTMS dans la phase de récupération.
L’efficacité a été démontrée dans les cas de la dépression résistante et des hallucinations auditivo-verbales non-répondantes aux traitements pharmacologiques. La rTMS pourrait être considérée aussi pour les patients (avec une dépression endogène), qui refusent un traitement pharmacologique.
Parmi d’autres conditions pour lesquels le traitement de rTMS peut être considéré, il y a aussi les acouphènes et les troubles anxieux. Pour les pathologies psychiatriques l’indication doit être discutée obligatoirement avec le médecin psychiatre référent du patient. Pour la fibromyalgie l’indication doit être discutée avec le rhumatologue référent du patient.

Nous sommes naturellement à votre disposition pour discuter de chaque patient les indications et le protocole de la rTMS.

Vous pouvez nous joindre à notre cabinet au 0041 22 9959744

Dr Antonio Carota et Dr André Menétrey

Pour d’autres informations, il faut visiter

http://tms-therapie.ch/fr/


 

Pubblicato in Headache, Neurorehabilitation, Psychiatry, Uncategorized | Lascia un commento

Dysprosody

The prosody is a communicative linguistic function, which results from the intonation, cadence, accent, and physical duration of the words. The prosody enhances the comprehension or the composed words, the basic emotions (rage, fear, sadness, surprise, disgust, pleasure), the subtle emotional aspects of the discourse (irony, sarcasm, deception, boredom, solace), and allows the differentiation of declarative, interrogative, and imperative phrases.

Thus, the expressive (affective) dysprosody is a suprasegmental deficit of language which should not be explained by a motor (dysarthria) or premotor (language apraxia) deficit, nor phonological or aphasic dysfunction (such as agrammatism and anomia). The patients with receptive dysprosody do not understand the emotional information of the phrases or the meaning of gesticulation.

Affective dysprosody could be an early predictor of post-stroke depression.Several studies on brain-damaged patients and normal subjects demonstrated the dominant role of the right hemisphere for prosody. In acute stroke settings, the assessment of dysprosody by bedside tests could help in localizing the lesion to the right hemisphere. Dysprosody, during epileptic seizures, has been linked to right hemisphere foci. The profile of anatomical correlation of prosodic syndromes (motor aprosodia for anterior and receptive dysprosody for posterior lesions) seems to parallel one of the aphasic syndromes of the left hemisphere.

Functional neuroimaging studies on normal subjects also provided a dichotomous scenario for linguistic functions such as the left hemisphere dominance for phonological and phonetic aspects versus the right hemisphere dominance for the emotional aspects.

Dysprosody might be amenable to behavioral treatments.

Pubblicato in Cognition and Behavior | Lascia un commento

A case of apraxic agraphia?

Apraxic agraphia is a very rare condition. The examiner should think about it when the patient shows normal linguistic capabilities (conversation is normal or almost normal) together with severely impaired writing. Apraxic agraphia is a peripheral writing disorder. Patients with apraxic agraphia have reduced ability to make the motor movements needed to write letters and sequence writting strokes. However, they usually have unimpaired capabilities of oral spelling. In the case of apraxic agraphia, writing is hesitant, imprecise and the disorder should not be explained by motor, sensory, extrapyramidal or cerebellar deficits and executive dysfunction (perseverations).
In case of stroke apraxic agraphia has been reported with frontal, parietal and thalamic lesions. Apraxic agraphia can manifest also with frontotemporal dementia and corticobasal syndrome.
Recently, I examined a 60 years old left-handed man, who had brain multiple ischemic stroke due to an hypercoagulability “trousseau’s syndrome”. The brain MRI showed 2 large lesions respectively of the left superficial territory of the posterior cerebral artery (occipital-temporal basal regions) and of the right parietal supramarginal gyrus.  Motor and sensory examination was normal.
This patient had normal linguistic output (normal conversation), mild denomination deficit, signs of visual apperceptive agnosia, alexia (letter-by-letter reading), and some features of apraxic agraphia. Oral spelling was spared. The patient’s writing was extremely low and hesitant as he did not remember how to do it.
An example of the patient’s writing on dictation is reported in the figure.
In this case, it is difficult to establish clinical-anatomical correlations (the same dysfunction is at the origin of both agraphia and alexia?), without further assessment but patients with atypical dominance could help in dissociating mechanisms of neurocognitive syndromes. Could you suggest your personal protocol to assess agraphia?

Pubblicato in Cognition and Behavior | Lascia un commento

Stimulate your cortex and eat less

BrainEating Eating disorders should raise up from dysfunction of neural processing in brain areas that are involved in emotional control, appetite regulation and body schema representations. In this context, eating disorders are located in the interface between the mind, body and the sense of self-agency.
It has been suggested that changes of the cognitive control exerted by the dorsolateral prefrontal cortex on the anterior ventral striatal pathway (a center predisposed to appetite regulation according to feelings of positive or negative reward) could trigger bulimic or anorexic behaviors.
One of the best evidence of such control cortical mechanisms on appetite regulation is the “Gourmand Syndrome” described by T. Landis and coll. Such a syndrome, which was identified in stroke patients with right hemisphere anterior lesions, consists of developing passion or manic thoughts for fine food and eating.
Even if the localizations and psychological mechanisms were globally known it would be still difficult to understand cortical changes in the brain in term of more specific circuits and neurotransmitters. To proceed to such a knowledge systematic studies with specific questionnaires or experimental cognitive eating paradigms (coupled with functional neuroimaging) should be conducted on patients with focal brain lesions or focal epilepsies.
Repetitive magnetic stimulation or transcranial direct current stimulation therapies, based on the result of those researches, would be tempted on patients (without brain lesions) and with eating disorders such as bulimia or anorexia or on patients with malnutrition or obesity

Pubblicato in Cognition and Behavior | 2 commenti

Cervical Spondylotic Myelopathy (CSM): Nothing is easy!

CSM_1Although CSM is a frequent cause of myelopathy, its natural history is not clear, the clinical and radiological diagnosis might be particularly difficult and approaches to treatments are not standardized nor based on evidence medicine.
CSM is a degenerative condition of the cervical spine (spondylosis, disk herniation, spur formation, degenerative changes of facet joints, longitudinal ligaments and ligamentum flavum) leading to narrowing of the spinal canal (AP diameter < 13 mm), and to myelopathy and radiculopathy.
First symptoms are chronic suboccipital and cervical pain and decreased neck mobility but patients come generally to medical examination when they manifest weakness and paresthesia of arms and gait difficulties. In typical cases, the neurologist appreciates, together with reduced cervical motility, the presence of radicular signs of the superior limbs and corticospinal signs of the inferior limbs.
It should be never forgotten that false-positive and false-negative MRI results occur frequently in patients with radiculopathy and that therefore clinical findings about root involvement are fundamental when evaluating for surgery. Furthermore and not rarely patients have tandem spinal stenosis (simultaneous cervical and lumbar stenosis). Thus, complete neurological examination is determinant.
MRI examination is needed for diagnosis as the MRI images might show both spinal canal stenosis and signs of myelopathy. MRI diffusion tensor imaging (DTI) could be also useful.
However, in doubtful cases it is important to remember that, there is also the contribution of dynamic factors such as the effect of movements of the cervical spine on the spinal cord, and of vascular factors (spinal artery deformation and compression with resulting spine ischemia and hypoxia). Thus, it would be also important, for therapeutic decisions, to include, besides structural factors, the presence of cervical spine instability. Flexion-extension Rx views may detect cervical instability. Even when the diagnosis of CSM is done, but the disease is mild, it is not clear what it is the correct time for surgery as the deterioration rate remains unknown (deterioration is probably rare) and there are no radiological features able to predict the outcome (except probably for large and extended MRI T2 medullary hyperintensities). However, when radiological and clinical features are both significant but the patient is too invalidated by the condition, it would be probably too late for surgery. However, the main goal of surgery should be to avoid the progression of the disease and, therefore, there should be objective signs of myelopathy with clnical, MRI and evoked potentials tests.
Somatosensory and motor evoked potential could be useful to find signs of medullary involvement in doubtful cases.
Important elements to favor surgery are age < 75, the clinical severity and speed of progression of neurological symptoms and signs. Clinical severity is better evaluated with the aid of the modified Japanese Orthopedic Association (mJOA) scoring system. Surgical intervention is considered for score > 12 or >16. A favorable outcome (confirming that surgery was the best option) would be do demonstrate, after surgery, the improvement of evoked potential in comparison to the condition before surgery.
Surgical approach could be extremely varied depending of the site(s) of stenosis and associated degenerative lesions of disks and articulations. There would not be substantial differences of outcome between anterior and posterior approaches. Anterior approaches include: discectomy without or with bone graft, cervical instrumentation. Posterior approaches include decompressive laminectomy and foraminotomy, hemilaminectomy and laminoplasty.
Perioperative and delayed complications (among which, dysphagia, infections and other medical problems but also worsening of myelopathy) are not rare, especially in older patients. The use of combined anterior-posterior procedures show the higher risks of complications. Pre-surgery evaluation should include also neurologists and internal medicine specialists.
Probably most cases of mild or moderate CSM will not deteriorate or require surgery if the patient follow a strict conservative treatment consisting of cervical exercises with physical therapists, postural learning with occupational therapists, using collar in case of pain exacerbation, avoiding risky activities, avoiding cervical manipulations and prolonged flexion of the neck. However, for these patients, the neurological survey should be accurate and timed.

Pubblicato in Spine | Lascia un commento

Neurologic mysteries: Mild Encephalopathy with Reversible Splenium lesion (MERS)

MERSMERS is not a so rare clinical entity even if epidemiological data are scanty; the clinical presentation is variable such as the etiologies of this syndrome. However, although the splenium lesion is usually quite big, these patients do not show signs of posterior callosal disconnection (such as alien hand, left hand agraphia or apraxia, tactile aphasia or anomia, agraphia without alexia). They show some form of encephalopathy (delirium, confusion, transient consciousness alteration) and few cases have been reported with relatively pure psychotic features.
Causes include metabolic or toxic conditions such as epileptic drug withdrawal, epileptic status, hyponatremia and/or hypoglycemia, vitamin deficiency or malnutrition, hemolytic uremic syndrome, thyroiditis, neuroleptic malignant syndrome, EBV or influentia virus encephalitis, Kawasaki disease, Marchiafava-Bignami’s disease (in this condition demyelination usually occurs in the central part of the corpus callosum).
The brain MRI establishes the diagnosis showing the typically large isolated spherical lesion in the splenium of the corpus callosum. This lesion is usually hyperintense in T2, FLAIR and DWI images, with low ADC values and does not show contrast enhancement. In circumstances that are more exceptional other similar lesions could be present on the periventricular regions in the sémiovale center.
Although MRI feature of MERS are specific, some differential diagnosis should be thought, such as lymphoma, multiple sclerosis, and stroke.
EEG does not provide further elements to diagnosis and it is often normal. LCR examination would be indicated in most cases especially if the patient is still symtpomatic.
There are several hypotheses on pathogenesis: transient intramyelinic edema after generalized seizure, hyponatremia axonal damage, oxidative stress, AED toxicity and vasogenic mechanisms. Actually, the splenium of the callosum body probably receives blood from multiple arteries and the hypothesis of watershed mechanisms is less probable. The multitude of causes producing this unique lesion suggest a common but not specific pathogenic mechanism. This mechanism should have some similarity, I suppose, with the one at the origin of the subcortical occipital MRI abnormalities observed with posterior reversible encephalopathy.
Although some authors advanced the possibility of IgG or steroid pulse therapies, there is no specific therapy for MERS as the condition is usually fully reversible.

Pubblicato in Cognition and Behavior, Neurologic mysteries | Lascia un commento

Micrographia

MicrographiaMicrographia is a frequent symptom of Parkinson’s disease, often one of the first. Its association with the disease is straightforward: 75-90% of patients with Parkinson show micrographia, the presence of micrographia allows the diagnosis of PD in almost all cases. In other less frequent disorders, micrographia is associated with other parkinsonisms (Huntington’s disease, Wilson’s disease, Progressive Supranuclear Palsy, Binswanger’s disease and so on), which have degenerative, genetic or vascular causes and which all impair basal ganglia circuits as it happens with PD.
Micrographia denotes a small handwriting, which generally is also slow and less accurate. Micrographia can be also detected by asking to the patient to write by air movements of the fingers. Patients with handwriting do also small drawings (see the watch in the figure).
Specific software on computer and tablets allows measuring accurately the handwriting characteristics (size, duration, speed and fluency). Such digital measures of handwriting would help formulating the diagnosis of PD in the earlier phases. It would be important for PD diagnosis, even, without any specific measure, to compare visually the actual handwriting of the patients with the previous examples of the years before.
Two variants are described, although, they often coexist in the same patient. “Constant micrographia” is the constantly reduced form of the small handwriting. This is the general and distinctive feature of the micrographia itself. On the other hand, “Progressive micrographia” is the tendency for handwriting to reduce progressively its size more and more in the course of writing. This corresponds to the general “sequence effect”, a well-known phenomenon in patients with PD or other parkinsonisms, consisting in progressively reducing the amplitude and fluency of motor sequences (parkinsonian gait festination and/or gait freezing are examples). Similar to the progressive micrographia, in the case of gait festination, the patient involuntarily moves with short, accelerating steps, often on tiptoe, with the trunk flexed forward and the legs flexed stiffly at the hips and knees.
The “constant micrographia” is the direct expression of the dysfunction of dopaminergic motor circuits of the basal ganglia and can improve with levodopa or by training in handwriting rehabilitation. Progressive micrographia is determined by the dysfunction of larger connectivity of brain areas (including cerebellum or parietal and motor/premotor cortical areas) and less responsive to dopamine or rehabilitative programs.
Micrographia should be assessed and measured with digital instruments in PD patients, not only for diagnostic purposes but also to monitor and quantify the improvement of patients with pharmacological and rehabilitative treatments.

Pubblicato in Cognition and Behavior, Parkinson Disease | Lascia un commento

Alzheimer’s disease: when the cure?

ALZHTo answer this question we should list some significant facts about the disease. There are 36 millions patients with the disease in the world, a diagnosis of Alzheimer disease is made every three seconds in the USA, the costs of the disease in the world are 600-800 billions of dollars and there is convergent evidence that indicates that the asymptomatic phase (before the onset of symptoms), last 15-20 years. Up to now, there is not a cure for the disease, although we know a lot on the genes and the proteins, which are involved.
The current available drugs (three acethylcholine-esterase inhibitors and one NMDA antagonist) show some efficacy only for 2/3 of patients and this effect lasts generally 6 months. Some studies regarding the effects of the association of these drugs are ongoing.
The CERE 110 is a phase I study that provided evidence that bilateral stereotactic administration of genetically engineered gene-therapy vector adeno-associated virus serotype 2 delivering NGF (AAV2-NGF)in to the nucleus basalis of Meynert is feasible, well-tolerated, and able to produce long-term, biologically active NGF expression. Phase II and III studies are needed.
EVP-6124 (Encenicline) is an α7 nicotinic acetylcholine receptor partial agonist under investigation for the symptomatic treatment of AD. Treatment with EVP-6124 in Phase I and II trials involving patients with mild-to-moderate AD was well tolerated and showed statistically significant improvements compared with placebo on cognitive and functional measures. Two Phase III studies under the title COGNITIV AD are ongoing.
Most phase III studies targeting Aß amyloid with monoclonal antibodies (Solanezumab, Crenezumab, Gantenerumab) did not show significant results. However, some positive trend n patients with mild or moderate disease was observed, data, which seems sufficient to extend these phase III trials to patients with the mildest form of the disease. Results will be available in few years.
The A4 trial will enroll normal individuals with amyloid accumulation, who are at increased risk for cognitive decline due to AD. 5000 clinically normal older individuals will be screened to identify those with increased amyloid accumulation on PET imaging; those individuals will be randomized into the anti-amyloid treatment arm with solanezumab or placebo arm. Individuals who do not show evidence of elevated amyloid accumulation may be eligible in an observation arm that will run parallel to the A4 treatment arm with identical cognitive assessments. A4 and LEARN study participants will be followed for 168 week treatment and observation periods.
The multicenter Dian-tu and API ADAD trials will enroll large samples of individuals who are carriers of different genetic variants of AD and will treat them with anti-amyloid monoclonal antibodies during the asymptomatic phase of the disease. The last two studies will give results after 2020.
Studies of drugs against secretases (MK/8931) are in phase II/III and results are expected on 2020.
Human studies on vaccines inducing immune response against Aß amyloid are still in phase I and II.
Therapies targeting tau proteins are for the moment only studied in animal models.
TTP488 is an orally active antagonist of RAGE (receptor for advanced glycation end-products). A phase II study did not provide significant results and showed concerns for safety of higher doses.
Pioglitazone, an insulin sensitizer of the thiazolidinedione class of peroxisome-proliferator activated receptor γ (PPAR-γ) agonists is under study on a phase III trial.
A Phase II study of saracatinib (AZD0530), a small molecule inhibitor with high potency for Src and Fyn, a family of kinases intervening in the synthesis of amiloid and tau, is ongoing.
In conclusion, there is nothing substantially new regarding a cure for the disease. Some optimism should be expressed for the future phase III studies including asymptomatic patients with the genetic variants of the disease and who will be trailed with monoclonal anti-amyloid antibodies. We have to wait still 5 years and probably even more for the results of such studies. However, it seems evident, from biological data we have, that Alzheimer disease is a multiple-mechanism disease requiring probably for a cure the combination of multiple drugs.

Pubblicato in Alzheimer | Lascia un commento

Reduplicative Paramnesia

REDPARAThis syndrome is quite rare and there are no specific epidemiological data. It can be observed in both psychiatric and neurologic settings. In my personal neurologic career, I probably met thirty patients with this condition, most of them after frontal or temporal stroke on the right hemisphere. Few patients had Lewy body disease or other forms of dementia. Other few patients showed the disorder after traumatic brain injury (with hemorrhagic lesions or contusions predominant on the right hemisphere).
Patients with reduplicative paramnesia believe firmly that a familiar place (generally their own house), has been duplicated and exists simultaneously in two or more locations.
Most patients I met believed that the hospital was actually their own house disguised into the hospital. Others were convinced that the hospital was located in another town than the actual (for example Geneva instead than Lausanne). Other patients indicated a sort of chimeric assimilation: finally, a fusion between the hospital and their house took place. These false believes about places cannot be resolved by providing obvious and logical evidence of the contrary. The strength of this delusion (usually explained by the patient with improbable explanations, so called confabulations) is the key clinical element for the diagnosis of reduplicative paramnesia. However, differently than patients with schizophrenia, neurologic patients with reduplicative paramnesia appear collaborative or show little concern for their condition and do not feel themselves completely menaced by the delusion. The exact neural mechanisms of reduplicative paramnesia are not completely understood. The patients with brain lesions generally show memory difficulties together with visuospatial deficits and defective reality checking. Furthermore, this syndrome requires some distorted sensation of familiarity, which finally arises the patients’ doubts on the identity of places. Finally, there is some kind of anosognosia and/or a general reduction of awareness. There are no specific rehabilitation protocols for the treatment of this condition. Some empirical cues are systematically given to the patients to enhance reality orientation. Rarely patients need some pharmacological treatments for the delusion (low doses neuroleptics).
Actually, I follow, as outpatient, an 80 years old woman with a relatively pure form of reduplicative paramnesia 6 months after a medial temporal lobe stroke on the right hemisphere. Actually, she believes that the some furniture of her own house has been removed and is still in the hospital where she had rehabilitation. Her standardized neurobehavioral assessment showed moderate memory visuospatial and executive deficits and the patient verbalizes (despite with mild confusion) her doubts on what is real or not in her thoughts on the location of the furniture. Some suggestions for further assessment and/or treatment?

Pubblicato in Cognition and Behavior, Stroke | 5 commenti