神经系统疾病

Clinical indications of amyloid imaging in atypical dementia remain unclear. We report a 66-year-old female without past psychiatric history who was hospitalized for auditory hallucinations and persecutory delusions associated with cognitive and motor deficits. Although psychotic symptoms resolved with antipsychotic treatment, cognitive and motor impairments remained. She further showed severe visuoconstructive and executive deficits, ideomotor apraxia, elements of Gerstmann's syndrome, bilateral agraphesthesia and discrete asymmetric motor deficits. Blood tests were unremarkable. Structural brain imaging revealed diffuse fronto-temporo-parietal atrophy, which was most severe in the parietal regions. Meanwhile, FDG-PET suggested asymmetrical fronto-temporoparietal hypometabolism, with sparing of the posterior cingulate gyrus. A diagnosis of possible corticobasal syndrome (CBS) was made. Amyloid-PET using the novel tracer NAV4694 was ordered, and revealed significant deposition of fibrillar amyloid (SUVR 2.05). The primary diagnosis was CBS with underlying Alzheimer pathology and treatment with an acetylcholinesterase inhibitor was initiated. Determination of underlying pathological CBS subtype is not simple even when based on extensive investigation including clinical presentation, atrophy patterns on MRI, and regional hypometabolism on FDG-PET. By contrast, amyloid imaging quickly confirmed Alzheimer pathology, and allowed rapid initiation of treatment in this complex case with early psychiatric symptoms. This case study illustrates the clinical utility of amyloid imaging in the setting of atypical cases seen in a tertiary memory clinic.

Status dystonicus is a rare, but truly a potentially life threating movement disorders emergency. Recognizing this condition requires awareness, and also knowledge of phenomenological and clinical characteristics. We describe here a patient who was recently diagnosed with this condition, and the clinical features as well as treatment options.

Background: OnabotulinumtoxinA has proven efficacious as a preventive treatment in patients with chronic migraine. However, non-response and tolerability problems were reported for some patients. This open-label study investigated long-term treatment with incobotulinumtoxinA in chronic migraine patients who were non-responsive to onabotulinumtoxinA injections.
Methods: Patients received pericranial injections of 200 U incobotulinumtoxinA (fixed dose) into 20 injection sites (10 U/site) using a dilution of 10 U/0.1 ml saline every three months for 18 months. They were subsequently switched back to onabotulinumtoxinA (200 U fixed dose into 20 injection sites with 10 U/site every 3 months) and were followed for another six months (length of study 24 months). The primary efficacy endpoints were monthly migraine episodes, pain intensity, the impact of migraine on functioning in daily life and consumption of pain relief drugs.
Results: Twenty-six patients (mean age 32 ± 4 years, 84.6% female) were included. IncobotulinumtoxinA significantly reduced mean monthly frequency of migraine episodes from 17.2 ± 2.1 to 4.3 ± 1.0 (p<0.05), pain intensity during these episodes from 9.3 ± 0.8 to 6.3 ± 0.9 (p<0.05) and consumption of acute pain medication from 32 ± 2 tablets to 5 ± 0.9 tablets after 18 months of treatment (all p<0.05). Patients’ daily functioning improved from severe (29 ± 3 points) to mild disability (8.2 ± 1.2; p<0.05). Six months after switching back to onabotulinumtoxinA, the frequency of monthly migraine episodes was 4.1 ± 1.0 (p<0.05), pain intensity 5.8 ± 0.9 (p<0.05), and consumption of acute pain medication 4 ± 0.9 tablets (p<0.05). Patients’ daily functioning was 8.2 ± 1.2 points (p<0.05; all p vs. baseline). Five mild adverse events were reported in the incobotulinumtoxinA treatment period.
Conclusions: Both botulinum toxin type A preparations were administered at a higher total dose at fewer injection sites in higher concentrations (10 U/0.1 ml) than previously described. This treatment regimen resulted in long-term benefits in incobotulinumtoxinA treated chronic migraine patients who were non-responsive to previous onabotulinumtoxinA injections. These improvements could be sustained after switching back to onabotulinumtoxinA. Treatment was well tolerated.

Parkinsonism- hyperpyrexia syndrome (PHS) is a neurological emergency that mimics neuroleptic malignant syndrome and sepsis. Abrupt cessation of anti parkinsons drugs, usually levodopa is responsible for this syndrome. Relative dopamine deficiency is proposed mechanism for PHS and replacement of dopaminergic drugs is the mainstay of treatment. We report a case who presented with manifestations of PHS after missing a single dose of levodopa.

Amyotrophic lateral sclerosis, commonly known as Lou Gehrig's disease, or simply ALS, is a neurodegenerative disease that slowly kills its victims within a few years after diagnosis. This is a complex disease in which every motor neuron in the body is killed over a relatively short time period - within 3 years or so, thus killing the victim. This disease can be classified into three main categories based on the neurological levels affected - bulbar onset, cervical onset, and lumbar onset (Mitchell and Borasio). Bulbar onset can further be divided into two subcategories - upper motor neuron (UMN) bulbar onset, and lower motor neuron (LMN) bulbar onset. The former involves loss of speech due to spastic dysarthia and the latter involves wasting of tongue tissue accompanied with flaccid dysarthia and dysphagia (Kiernan et al.,). Bulbar onset occurs first about one third of the time (Gordon). Cervical onset involves loss of function in upper limbs and can include both upper motor neurons and lower motor neurons. In contrast, lumbar onset involves weakening of lower limbs, including feet (Mitchell and Borasio). Rilutek was first FDA approved to treat ALS almost a decade ago. Since then, several studies have shown that Rilutek may not be so effective at prolonging life in patients with ALS. In recent years, stem cells have become a subject of great debate among the scientific community due to their implications in curing diseases like ALS. Stem cells have been shown in clinical trials to prolong life in patients in ALS even more than Rilutek has. They have also shown to control glutamate uptake in transgenic mice with the SOD1 gene, which is promising for new treatments in ALS patients. Although Rilutek has also shown to control glutamate uptake, stem cells may have a better effect

Molecular hydrogen (H2) has been reported to be effective for a variety of disorders and its effect has been ascribed to a selective scavenge of hydroxyl radicals (•OH) at the beginning. Consumption of H2 was either by inhalation, drinking H2-containing water (H2 water), or infusion of H2-containing saline. Among various disorders, animal model of ischemic injury and Parkinson’s disease showed significant amelioration after H2 treatment. The mechanism of neuroprotection, however, is not simple. Multiple mechanisms may exist to produce acute and chronic effect. For chronic effect, H2-induced neuroprotection takes several days to develop and lasted several days, suggesting that H2 may work as a modulator of signal transduction as indicated by allergy model. The evidence that drinking H2 water was the most effective way rather than inhaling H2 in Parkinson’s disease model animal led not lead to the finding that H2 induces ghrelin production and release from the stomach by activating β1 adrenergic receptors. The distinct mechanism due to the brain-stomach connection may help to understand the broad spectrum of H2 function. In addition, clinical trials have shown promising results.

Cerebral palsy is one of the most common chronic childhood disorders, occurring in 2-2.5 of every 1000 live births; it is a persistent disorder of movement and posture caused by non-progressive lesions of the immature brain. Epilepsy is an important problem in children with cerebral palsy, with past studies showing an average incidence of epilepsy in about one-third in cases of cerebral palsy. Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate seizures, common in patients who have other factors (such as cerebral palsy) that are associated with a very high likelihood of a persistently lowered seizure threshold and therefore a high recurrence risk. A few studies were conducted in the past analyzing the correlation of cerebral palsy and epilepsy in children; however, no study has been conducted yet evaluating the evolution of cerebral palsy in the transition from childhood to adulthood, and how the incidence, prevalence and clinical course of epilepsy change in such a time lapse. Furthermore, no studies yet evaluated the classification of seizures in this adult population, their correlations to cerebral palsy and its degrees of severity, and associated secondary health conditions, causing epidemiological data to be lacking in that regard. This aim of this manuscript is to introduce to the reader the current evidence regarding epilepsy in adults with cerebral palsy, as well as present recommendations for future research.

Objective: To delineate the profile of pediatric patients with stroke in Western Rajasthan.

Methods: This was a hospital based prospective follow-up study carried out over a period of one year. In the study all admitted patients (6 m-18 years old) fulfilling the both clinical and radiological criteria (CT Scan/MRI) for stroke were enrolled (n=50). Epidemiological profile, clinical parameters and complications of these 50 patients were analyzed.

Results: 50 patients of stroke (as per case definition) were admitted in our institute over a period of one year out of which 64% were males (n= 32) and 36% were females(n=18) with a ratio of 1.78:1. 48% patients were in age group of 6 to 24 months (n=24); with a overall mean age of presentation of 52.8 ± 51.0 months. 74% (n=37) of the patients had Arterial Ischemic stroke (AIS); 8% (n=4) had Cerebral Sinus Venous Thrombosis (CSVT); 6% (n=3) had hemorrhagic stroke; 12% (n=6) had AIS coexisting with CSVT and hemorrhagic stroke. AIS was most commonly seen in age group of 6-24 months of age (45.95%); CSVT was most commonly seen among children of 60-120 months (50%); Combined type of stroke was seen only in patients between age group of 6-60 months. Anterior circulation was most common circulation involved in patients of stroke at all ages (63%), followed in frequency by combination of both anterior and posterior circulation (28.3%) and posterior circulation alone (8.7%). Involvement of both hemispheres of the brain together was slightly more common (36%) than either right side alone (34%) or left side alone (30%). Middle cerebral artery (MCA) territory stroke was the most common territory to be involved at all ages (45.6%). Isolated Posterior cerebral artery (PCA) & Internal carotid artery (ICA) territory stroke were more common (28.6%) in children >120 months. Involvement of more than one vessel territory was seen in children between 6-24 months (20.8%) and 24-60 months (44.4%) of age. Seizures were reported in 80% (n=40) of children amongst which 45% (n=18) had generalized seizures and 55% (n=22) had focal seizures. In patients with AIS, focal seizures were more common (54%) than generalized seizures. Patients who had CSVT type of stroke, only 50% patients had seizures. All hemorrhagic stroke patients presented with generalized seizure. Impairment of consciousness was seen in 50% of stroke patients. Consciousness was most commonly affected in patients of hemorrhagic stroke (100%). Co-morbid conditions were seen in 58% patients and Neurotuberculosis was the most common (22%) co-morbid condition associated with patients of stroke. Hemiparesis was the most common (66%) mode of presentation followed by facial palsy (26%). 26% (n=13) patients of stroke had no neurological deficit at the time of discharge and 36% (n=18) patients had mild to moderate neurological deficit, 30% (n=15) had severe deficit and 8% (n=4) patients expired.

Conclusion: Thus stroke in childhood is an important issue to be addressed as it is not uncommon as previously thought and is an important cause of morbidity and mortality. Despite excellent advances in clinical care and increasing availability of infrastructure, newer medicines and technology, the exact clinic-epidemiologic profile of both adult and pediatric stroke in Western Rajasthan is still not available. The current study has therefore been designed to evaluate the clinic-radiologic profile of pediatric stroke and to see whether stroke profile, mode of presentation and outcome of

We present four patients with first episode psychosis who were also negative to other clinical evaluations including thyroid functioning. Their psychotic symptoms were suspected to be induced by the arachnoid cyst and it was controlled by moderate dose antipsychotic administration. The diagnoses of these cases were atypical affective disorder, schizophrenia (paranoid type), delusional disorder (jealous type), atypical psychosis. All of the cases manifested a variety of delusions of persecution, mystic, megalomaniac, jealousy. Neurosurgical examinations only revealed that patients needed to follow-up.

Background: Migraine is a chronic neurological disorder characterized by headaches along with several physiological and autonomic nervous system symptoms. Research suggests that migraine is a result of multi-gene mutation in combination with psycho-social and environmental factors.
Methods: Mutated mammalian serotonin hydroxytryptamine receptor 2 (HTR2) implicated as factor causing migraine were retrieved from the National Centre for Biotechnology Information (NCBI), its 3D structure were determined by homology modelling. The 3D structures of phyto-compounds (from Ayurvedic herbs) were retrieved from various databases. The pharmacophore hypothesis was generated for the existing ligands and the phytocompounds were screened against the generated pharmocophoric hypothesis. Ligands were shortlisted based on their fitness score. The selected phytocompounds were screened against HTR2 receptor.
Results: The phytocompound having the best docking score and most interactions with the receptor are validated using receptor-ligand binding assay studies with HTR2 receptor in-vitro.
Conclusion: Phytocompounds selected as per receptor-ligand binding assay studies.

Background: Normal Pressure Hydrocephalus (NPH) is a neurological disorder caused by excessive accumulation of cerebrospinal fluid (CSF) with resultant pressure to the brain. It is a rare syndrome usually found in the sixth and seventh decades of life. The condition is often misdiagnosed because of its non specific symptoms and chronic nature, but recently NPH is receiving a great deal of media and medical attention, leading to a more efficient and timely diagnosis and treatment.
Methods: This is a case report of a 40 year old widow, who presented with eight weeks history of recurrent vomiting, fearfulness and withdrawal to self following a traumatic experience. Prior to above presentation patient was normal and stable with no past history of physical or mental illness.
Results: Clinical features met the criteria for diagnosis of Post Traumatic Stress Disorder following a traumatic experience. Clinical examination and investigations including (skull xray) conducted were all within normal limits. Two weeks into admission patient developed seizure, facial nerve palsy, and progressive weakness of lower limbs which warranted further investigation. The patient had Computer Tomography scan which revealed NPH, she had a ventro-peritoneal shunt at the neurosurgical unit of the hospital.
Conclusion: Despite the advances in the diagnosis and treatment of hydrocephalus, there is still much to be learned about hydrocephalus and the subtle forms it can take. Even though it is more common in infants and elderly, it can occur at any time in life and as a result of a variety of causes.

High-frequency oscillations are changes in electrical potentials in the EEG in frequencies above 80 Hz. In recent years it was discovered that these oscillations have great importance both in physiologic processes and as an expression of epileptogenic areas in the cerebral cortex. The measurement of these oscillations via depth-electrodes during pre-surgical evaluation of epilepsy patients has been proven to be an important tool in improving the ability to delineate exactly the area which should be resected, in case such an operation is feasible. New technical developments enable the acquisition of high-frequency oscillations from scalp electrodes as well, which could have great importance in brain research and care of epilepsy patients in the future.

According to the recent World Health Organization’s large-scale studies, about a third of the adult worldwide population suوٴer from a mental disorder such as depression, anxiety and schizophrenia. If also taken together with neurological disorders, such as dementia and stroke, these “disorders of the brain” account for 13% of the global disease burden. Нis surpasses both cardiovascular diseases (5%) and cancer (10%). Countries with the highest rate of burden >650 Disability Adjusted Life Years [DALYs] per 100,000 population included the USA, UK, Russia, and Australia. Нe annual cost of taking care of patients with neurological disorders in the US alone approaches $400B of which $200B is for Alzheimer patients. We believe that this cost in 10 years could reach to $1T in the US alone. In China 975,000 people die annually from brain trauma alone and the Alzheimer population in the Australasia corridor is rapidly on the rise. Нus, European Union, China, India, Japan and Australia are not immune from such high cost of healthcare despite having 100% government insurance

Mollaret's meningitis is a rare disease characterized by recurrent self-limiting episodes of lymphocytic meningitis, mainly linked to viral infections (Herpes Simplex Virus and Epstein Barr Virus), and to a lesser extent to autoimmune diseases, alternating with periods in which the patient is asymptomatic.

The myelomeningocele (MMC) is a failure in embryo development during the fourth week of gestation, being the most common form of spinal dysraphism characterized by a protrusion of the meninges, roots and spinal cord by a bony midline defect [1,2]. The MMC treatment is surgical and should be implemented as soon as possible in order to reduce the exposure of the neural tissue and consequently the risk of infections and worsening of neurological deficits [3,4]. The incidence of hydrocephalus is higher in cases of neonates whose surgical repair of MMC is done after the child's birth (postnatal repair of myelomeningocele), when compared to the group of children undergoing intrauterine surgery [4]. For children whose correction and closing of the MMC is still done during intrauterine life (known as intrauterine fetal open surgery; (Figure 1), the incidence of hydrocephalus can be reduced by 50%, as well as motor deficits [4]. In Figure 1 we show the image of the first fetal neurosurgery performed in the northeast region of Brazil, with excellent result [5].

The blood brain barrier (BBB) refers to the complex anatomical barrier in the brain composed of endothelial cells, astroglia, pericytes, perivascular macrophages and basal lamina. Its selectivity controls the entry of substances into the Central Nervous System (CNS) [1]. BBB disruption affects neurodegeneration [2] and in some cases can be harnessed to deliver intravenous therapeutics to the CNS. Canavan’s disease (CD) is an autosomal recessive neurodegenerative disease caused by lack of aspartoacylase that causes extensive demyelination.

Sliver stain of olfactory bulb tissue section in a 74 year old male with a clinical history of Alzheimer’s, congestive heart failure, and pneumonia. A. A 10X magnification for the area containing the anterior olfactory nucleus (AON) (astercs). B. A 20X of the same area showing neurofibrillary tangles (arrow) and neuropil threads (double arrow). The autopsy showed definite Alzheimer’s disease (C on CERAD scale). Neuritic plaques identified in inferior parietal and parahippocampal areas from this patient. There were moderate tangles in peristriate and frontal cortex, and neuropil threads were prominent in mesial temporal structures and frontal cortex.

A 56-year-old woman complained of a three-month history of progressive clumsiness in her left hand. She reported fine hand movements to have become slow and uncoordinated, thus leading to moderate difficulty in dressing and working as a beautician. A neurological examination revealed rigidity in her left limb. No obvious tremors were noted. She was treated with L-dopa (300 mg/day), which provided substantial symptomatic benefits. MRI showed the putamen to have a normal morphology and size, and no areas of any altered signals were observed in the brain parenchyma (Figure 1A). Positron emission tomography (PET) with an aromatic L-amino acid decarboxylase (AADC) tracer, 6-[18F] fluoro-L-m-tyrosine (FMT) showed a reduced uptake in the posterior part of the right putamen.

Diagnosing spinal epidural abscess can be challenging. Patients may not present with the classic triad of fever, neurological deficits, and back pain, resulting in delayed diagnosis and treatment. We present a patient with back and abdominal pain, atrial fibrillation, and lactic acidosis.