1/9. Hypertonia, hyperreflexia, and excessive startle response in a neonate.Following an uneventful gestation, a newborn girl presented with hypertonia, hyperreflexia, tremor, and excessive startle response. nose tap elicited a dramatic head recoil. Her mother had similar symptoms beginning as a child that improved but persisted into adulthood. In addition, several members of mother's family died unexpectedly in infancy. Hypertonia in the newborn period indicates central nervous system dysfunction of several possible causes, most of which are associated with severe cognitive deficits and limited neurological development.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/9. cyproheptadine for intrathecal baclofen withdrawal.OBJECTIVE: To evaluate the efficacy of cyproheptadine in the management of acute intrathecal baclofen (ITB) withdrawal. DESIGN: Descriptive case series. SETTING: University hospital with a comprehensive in- and outpatient rehabilitation center. PARTICIPANTS: Four patients (3 with spinal cord injury, 1 with cerebral palsy) with implanted ITB infusion pumps for treatment of severe spasticity, who had ITB withdrawal syndrome because of interruption of ITB infusion. INTERVENTIONS: patients were treated with 4 to 8mg of cyproheptadine by mouth every 6 to 8 hours, 5 to 10mg of diazepam by mouth every 6 to 12 hours, 10 to 20mg of baclofen by mouth every 6 hours, and ITB boluses in some cases. MAIN OUTCOME MEASURES: Clinical signs and symptoms of ITB withdrawal of varying severity were assessed by vital signs (temperature, heart rate), physical examination (reflexes, tone, clonus), and patient report of symptoms (itching, nausea, headache, malaise). RESULTS: The patients in our series improved significantly when the serotonin antagonist cyproheptadine was added to their regimens. fever dropped at least 1.5 degrees C, and heart rate dropped from rates of 120 to 140 to less than 100bpm. Reflexes, tone, and myoclonus also decreased. patients reported dramatic reduction in itching after cyproheptadine. These changes were associated temporally with cyproheptadine dosing. DISCUSSION: Acute ITB withdrawal syndrome occurs frequently in cases of malfunctioning intrathecal infusion pumps or catheters. The syndrome commonly presents with pruritus and increased muscle tone. It can progress rapidly to high fever, altered mental status, seizures, profound muscle rigidity, rhabdomyolysis, brain injury, and death. Current therapy with oral baclofen and benzodiazepines is useful but has variable success, particularly in severe cases. We note that ITB withdrawal is similar to serotonergic syndromes, such as in overdoses of selective serotonin reuptake inhibitors or the popular drug of abuse 3,4-methylenedioxymethamphetamine (Ecstasy). We postulate that ITB withdrawal may be a form of serotonergic syndrome that occurs from loss of gamma-aminobutyric acid B receptor-mediated presynaptic inhibition of serotonin. CONCLUSION: cyproheptadine may be a useful adjunct to baclofen and benzodiazepines in the management of acute ITB withdrawal syndrome.- - - - - - - - - - ranking = 0.16640310244624keywords = brain (Clic here for more details about this article) |
3/9. frontal lobe dysfunction in sporadic hyperekplexia--case study and literature review.Hyperekplexia (HE), or startle disease, is usually a familial disorder associated with mutations in the glycine receptor alpha1 subunit gene (GLRA1), characterised by exaggerated startle reactions to unexpected auditory, somaesthetic and visual stimuli. Non-familial cases may be idiopathic, or associated with pathology usually in the brainstem or rarely in the supratentorial compartment. The pathophysiological basis of HE is unclear.We report the case of a 40-year-old woman presenting with excessive startle response to unexpected stimuli and falls since the age of 16 years. There was no family history. She was initially diagnosed with epilepsy and started on phenytoin with no resolution of her symptoms. Clinical examination revealed hyperreflexia and an insecure broad-based gait but no other abnormalities. Routine comprehensive neuropsychological assessment revealed below average intelligence with signs of frontal lobe dysfunction. EEG showed non-specific abnormalities in the right frontal and central regions. A (99m)Tc-HMPAO SPET scan revealed hypoperfusion in the frontal (worse on the right) and temporal lobes and to a lesser extent in the basal ganglia. MRI was normal, as well as blood and CSF tests. No mutations were found in a genetic analysis of GLRA1. The patient improved partially with treatment by clonazepam.The localisation of the clinical and neuropsychological findings accord with the EEG and SPET scan abnormalities in our patient and corroborates previous reports. Appropriate neuropsychological testing and functional imaging enable more accurate delineation of the clinical phenotype of this rare disorder.- - - - - - - - - - ranking = 0.16640310244624keywords = brain (Clic here for more details about this article) |
4/9. Hyperekplexia and sudden neonatal death.Fifteen patients with hyperekplexia were identified in 3 families; diagnostic clinical characteristics were defined which allowed for early recognition and treatment. During the first 24 hours of life, spontaneous apnea and sluggish feeding effort were observed. After the first 24 hours, surviving infants exhibited the hyperekplexic startle response to nose tapping. This startle response is characterized by sudden muscular rigidity, feeding-induced oropharyngeal incoordination, and poor air exchange often with apnea, persisting with repetitive nose tapping. Untreated infants experienced recurring apnea until 1 year of age. Three of 15 patients died unexpectedly during the neonatal period. patients treated with clonazepam (0.1-0.2 mg/kg/day) had no serious apneic episodes and startle reflexes were diminished. The pathophysiologic mechanism for hyperekplexia remains obscure. Electroencephalographic studies were consistently normal. The response to and tolerance of benzodiazepines are striking in newborns and infants and suggest an aberrant central nervous system reflex as the etiology; therefore, hyperekplexia should be considered in the evaluation of neonates and infants with apnea, aspiration pneumonia, episodic muscular rigidity, hyperexcitability, and near-miss sudden infant death syndrome. The need for immediate monitoring of at-risk infants, observation for signs of hyperekplexia, and initiation of clonazepam in these patients are emphasized. Hyperekplexic startle response to nose tapping should be included in the routine examination of all newborns.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/9. An unusual case of dantrolene sodium-induced urinary retention in post-traumatic minimally responsive state.INTRODUCTION: An unusual case of urinary retention is reported occurring during the inpatient rehabilitation of minimally responsive state (MRS) following severe traumatic brain injury (TBI). CLINICAL PICTURE: Urodynamic evaluation showed detrusor acontractility and subsequent management involved intermittent bladder catheterization and treatment of a single urinary tract infection. TREATMENT: Factors contributing to her bladder paralysis included severe immobility, tetraplegia and treatment of diffuse spasticity with oral dantrolene sodium 250 mg per day. constipation, diabetes and spinal cord injury were absent. In addition, she received unilateral partial sciatic neurolysis with 50% alcohol for severe knee flexor spasticity and intra-muscular Botulinum toxin A to both spastic upper limbs. OUTCOME: Bladder acontractility resolved completely when dantrolene was reduced with subsequent achievement of a catheter-free status and small post-void residual volumes. Repeat urodynamics showed spontaneous detrusor contractions. CONCLUSION: A discussion of possible aetiologic factors for detrusor acontracility following TBI is presented including a brief review of the literature.- - - - - - - - - - ranking = 0.16640310244624keywords = brain (Clic here for more details about this article) |
6/9. vertigo due to whiplash injury: a neurotological approach.A series of examinations were carried out to elucidate the characteristics of vertigo due to whiplash injury. In patients with whiplash injury, there are over-excitation of the cervical and/or the lumbar proprioceptors on one hand, and dysfunction of the central nervous system, such as the hypothalamus, the brain stem and the cerebellum, on the other hand. These two etiological factors induce disequilibrium by a trigger-and-target relationship in which the above proprioceptors act as a trigger and the central nervous system acts as a target. This postulate is applicable to the explanation of aural vertigo following whiplash injury. Autonomic reflexes in patients with whiplash injury can be explained not only as due to over-excitation of the cervical sympathetic nerves, but also to that of the cervical and lumbar proprioceptors. In other words, these reflexes are considered as being the proprio-autonomic reflexes. These reflexes are more evident in patients with cervical pain. Cerebellar symptoms can be manifested by over-excitation of the cervical and lumbar proprioceptors. These symptoms are more evident in patients with lumbar pain. The above differences in the autonomic reflexes and cerebellar symptoms of patients with cervical and/or lumbar pains can be explained on the basis of known fiber connections in the central nervous system. Hypertonicity of the cervical and lumbar erector muscles in patients with whiplash injury can be explained not only as due to over-excitation of gamma fibers, but also to that of sympathetic nerves in these muscles. This hypertonicity affects the central nervous system, causing disequilibrium following whiplash injury.- - - - - - - - - - ranking = 4.1664031024462keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
7/9. Discordant infantile encephalopathy with symmetrical thalamic calcifications in identical twins.Connatal thalamic calcifications in apparently uneventful pregnancies have been described in various case reports and in a single report in two sibs. On the other hand, this lesion is known to occur after a hypoxic-ischemic accident in the immature brain. In the present report infantile encephalopathy with symmetrical thalamic calcifications is observed in one sib of a monozygous twin pair. This observation adds to the evidence that the condition is acquired, most probably on the basis of hypoxia-ischemia, and provides strong evidence against autosomal-recessive inheritance.- - - - - - - - - - ranking = 0.16640310244624keywords = brain (Clic here for more details about this article) |
8/9. Neuroaxonal dystrophy at birth with hypertonicity and basal ganglia mineralization.A full-term male infant exhibited rigidity of all extremities with hyperreflexia beginning soon after birth and lasting until his death at age 6 months. Head circumference remained at the 25th to 50th percentile. Distinct sleep-wake cycles and responsiveness to visual, auditory, and tactile stimuli developed. Metabolic studies, skin biopsy, electroencephalography, and electromyography produced normal results. Head computed tomographic and magnetic resonance imaging scans revealed mineralization of the basal ganglia and thalamus. Muscle and nerve biopsy results were consistent with axonal dystrophy. autopsy showed widespread neuronal loss, with reactive gliosis, marked in the globus pallidus and brainstem reticulate core; spheroids in globus pallidus, nucleus cuneatus, and upper cervical cord; and mineralized neurons in the inner division of globus pallidus and thalamus. Neonatal hypertonia, rapid progression, and mineralization of the basal ganglia are unusual features of neuroaxonal dystrophy exhibited in this case.- - - - - - - - - - ranking = 0.16640310244624keywords = brain (Clic here for more details about this article) |
9/9. Botulinum toxin treatment of lumbrical spasticity: a brief report.Botulinum toxin A has been used to treat wrist and finger spasticity mainly through injection of the forearm flexor muscles. This case study describes its first reported use in managing spastic lumbricals of the hand. A 19-year-old male had significant flexion deformity and hypertonicity of the left wrist and hand, particularly the second through fifth metacarpophalangeal joints, after traumatic brain injury. By using the 0-4 Ashworth scale, spasticity of the lumbricals across the second to fourth metacarpophalangeal joints was rated 2, with persistent clonus of the finger flexors as confirmed by electromyography to the middle and ring fingers, even after botulinum toxin A injection of the flexor digitorum sublimis and profundus muscles. By using the electromyography-guided technique, botulinum toxin A was injected into the first lumbrical of the index finger (12 units), second and third lumbricals of the middle and ring fingers, respectively (15 units each), and fourth lumbrical of the little finger (10 units). At follow-up, clinical and electromyographic examination revealed a significant reduction in tone and clonus of the injected lumbricals. Ashworth scores of the lumbricals from the index to little finger improved to 1. Botulinum toxin A injection of the lumbricals can be beneficial in managing spasticity of these muscles. It is well tolerated and effective at doses of 10 to 15 units. Lumbrical injection of botulinum toxin A is a useful adjunct in our percutaneous armamentarium for managing the spastic hand.- - - - - - - - - - ranking = 0.16640310244624keywords = brain (Clic here for more details about this article) |