A Girl from Qatar with Post-Infantile Acquired Cerebral Palsy Caused By Submersion Injury: A Rare Etiology and a Therapeutic-Challenge.

Background: Cerebral palsy is a heterogeneous disorder that can cause a lifelong disability that is associated with a non-progressive damage in the brain. It is commonly caused by antenatal, perinatal, early postnatal and neonatal conditions. However, post-neonatal cases of acquired cerebral palsy have also been reported, and were commonly caused by infection. Patients and Methods: The family of a girl from Qatar, who developed severe cerebral palsy caused by submersion injury, consulted us about the possible therapies for her condition. Clinical picture and brain imaging abnormalities are described, and the relevant literatures were reviewed with the aim of suggesting possible evidence-based therapies. Results: At the age of 23 months, a previously healthy girl developed anoxic encephalopathy after experiencing submersion injury. MRI showed evidence of significant hypoxic ischemic injury primarily affecting the deep grey matter, hippocami, mid-brain and the posterior cortex. EEG showed diffuse slowness of cerebral activity and diffuse attenuation of the background without no epileptic abnormalities suggesting diffuse encephalopathy resulting from diffuse cortical injury. At the about age of three and half years, her family consulted us about her condition as she was still showing no awareness to the environment, showing no significant spontaneous movements. She had poor head control. Unable to sit or stand alone, and had a flexed posture. She was on levetiracetam (Keppra), diazepam, and baclofen 30 mg daily. She was still having tracheotomy, and was fed through gastrostomy tube. Conclusion: In this paper, the rare occurrence of severe post-infantile cerebral palsy is described. Emphasis is made on the possibility of using evidence-based multi-factorial therapies in cerebral palsy.

A Girl from Qatar with Post-Infantile Acquired Cerebral Palsy Caused By Submersion Injury: A Rare Etiology and a Therapeutic Challenge

Background: Cerebral palsy is a heterogeneous disorder that can cause a lifelong disability that is associated with a non-progressive damage in the brain. It is commonly caused by antenatal, perinatal, early postnatal and neonatal conditions. However, post-neonatal cases of acquired cerebral palsy have also been reported, and were commonly caused by infection. Patients and methods: The family of a girl from Qatar, who developed severe cerebral palsy caused by submersion injury, consulted us about the possible therapies for her condition. Clinical picture and brain imaging abnormalities are described, and the relevant literatures were reviewed with the aim of suggesting possible evidence-based therapies. Results: At the age of 23 months, a previously healthy girl developed anoxic encephalopathy after experiencing submersion injury. MRI showed evidence of significant hypoxic ischemic injury primarily affecting the deep grey matter, hippocami, mid-brain and the posterior cortex. EEG showed diffuse slowness of cerebral activity and diffuse attenuation of the background without no epileptic abnormalities suggesting diffuse encephalopathy resulting from diffuse cortical injury. At the about age of three and half years, her family consulted us about her condition as she was still showing no awareness to the environment, showing no significant spontaneous movements. She had poor head control. Unable to sit or stand alone, and had a flexed posture. She was on levetiracetam (Keppra), diazepam, and baclofen 30 mg daily. She was still having tracheotomy, and was fed through gastrostomy tube. Conclusion: In this paper, the rare occurrence of severe post-infantile cerebral palsy is described. Emphasis is made on the possibility of using evidence-based multi-factorial therapies in cerebral palsy.

Unfortunate Case of Hypoglycemia Induced Seizures Resulting in Anoxic Encephalopathy

Background: Severe hypoglycemia in patient with diabetes mellitus can manifest as seizures and coma, most commonly occurring after excessive insulin administration. Clinical Case: A 35-year-old woman with uncontrolled type 1 diabetes mellitus on basal-bolus insulin, complicated by gastroparesis, hypoglycemia unawareness, and frequent hypoglycemic episodes due to a tendency to give more than the recommended amount of insulin, who presented with seizures and diffuse brain edema as severe manifestations of profound hypoglycemia. Hemoglobin A1c was 7.3% but patient had wide variation in blood glucose (40- 300 mmol/L) on her glucometer. Patient had initially self-treated overnight hypoglycemic symptoms by drinking soda and then fell asleep without re-checking her blood sugar. She was then unresponsive in the morning. Patient’s husband reported that there was no glucagon at home since he used the last one and did not refill. On EMS arrival, patient had a blood glucose of 25, for which she received dextrose and glucagon with improvement of blood glucose but no change in mentation. En route to the hospital, patient developed tonic-clonic seizures and decerebrate posturing. She received Ativan and was intubated for airway protection. On exam, she had bilateral dilated sluggishly reactive pupils, eyes opened spontaneously but did not track, and limbs moved spontaneously but there was no purposeful movement. Initial CT head without contrast showed significant diffuse brain edema. Repeat MRI brain with and without contrast showed bilateral basal ganglia diffusion restriction with associated T2 and FLAIR hyperintense signal, suggestive of toxic-metabolic etiology including hypoglycemia. Video EEG showed findings consistent with anoxic encephalopathy. Patient received IV mannitol and IV dexamethasone for cerebral edema and Keppra for seizure prophylaxis, but was unable to be weaned from the ventilator and had to undergo tracheostomy and PEG tube placement, and was eventually discharged to inpatient rehabilitation. Conclusion: This case highlights the dangers of accidental overcorrection of high blood sugar with short-acting insulin and not appropriately treating hypoglycemia, which can lead to irreversible brain injury due to prolonged hypoglycemia. This unfortunate case highlights the importance of educating patients with diabetes mellitus on insulin how to appropriately manage low sugars to avoid such outcomes.

Athetosis and Dystonia

Athetosis is characterized by irregular, slow, writhing, bizarre movements seen in hands and feet. Athetosis is classified as minor athetosis and major athetosis based on its magnitude. Minor athetosis is seen in patients with mild cerebral palsy. Major athetosis is caused by organic lesions of the striatum, including cerebral palsy, as a residual state of encephalitis, and after anoxic encephalopathy. In these conditions, athetosis is commonly seen in combination with dystonia. Dystonia is characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive movements or postures or both. In many cases of dystonia, the affected part of the body shows an abnormal writhing posture in the resting condition, and slow, writhing involuntary movements are superimposed on the abnormal posture with voluntary movement. For childhood-onset patients, dystonia is most commonly generalized; in adults, typically there is focal dystonia. Focal dystonia includes blepharospasm, cervical dystonia, writer’s cramp, and musician’s cramp. It is often task-specific and can be treated with local botulinum toxin injection. Generalized dystonia is caused by long use of neuroleptic drugs (tardive dystonia) and a variety of neurodegenerative diseases.

Past Life Experiences and Neurological Recovery: The Role of Cognitive Reserve in the Rehabilitation of Severe Post-Anoxic Encephalopathy and Traumatic Brain Injury

Objective:Patients with an equivalent clinical background may show unexpected interindividual differences in their outcome. The cognitive reserve (CR) model has been proposed to account for such discrepancies, but its role after acquired severe injuries is still being debated. We hypothesize that inappropriate investigative methods might have been used when dealing with severe patients, which have very likely reduced the possibility of observing meaningful influences in recovery from severe traumas.Methods:To overcome this issue, the potential neuroprotective role of CR was investigated, considering a wider spectrum of clinical symptoms ranging from low-level brain stem functions necessary for life to more complex motor and cognitive skills. In the present study, data from 50 severe patients, 20 suffering from post-anoxic encephalopathy (PAE) and 30 with traumatic brain injury (TBI), were collected and retrospectively analyzed.Results:We found that CR, diagnosis, time of hospitalization, and their interaction had an effect on the clinical indexes. When the predictive power of CR was investigated by means of two machine learning classifier algorithms, CR, together with age, emerged as the strongest factor in discriminating between patients who reached or did not reach successful recovery.Conclusions:Overall, the present study highlights a possible role of CR in shaping the recovery of severe patients suffering from either PAE or TBI. The practical implications underlying the need to routinely considered CR in the clinical practice are discussed.

SSEP retains its value as predictor of poor outcome following cardiac arrest in the era of therapeutic hypothermia

Objectives To re-evaluate the role of median nerve somatosensory evoked potentials (SSEPs) and bilateral loss of the N20 cortical wave as a predictor of unfavorable outcome in comatose patients following cardiac arrest (CA) in the therapeutic hypothermia (TH) era. Methods Review the results and conclusions drawn from isolated case reports and small series of comatose patients following CA in which the bilateral absence of N20 response has been associated with recovery, and evaluate the proposal that SSEP can no longer be considered a reliable and accurate predictor of unfavorable neurologic outcome. Results There are many methodological limitations in those patients reported in the literature with severe post anoxic encephalopathy who recover despite having lost their N20 cortical potential. These limitations include lack of sufficient clinical and neurologic data, severe core body hypothermia, specifics of electrophysiologic testing, technical issues such as background noise artifacts, flawed interpretations sometimes related to interobserver inconsistency, and the extreme variability in interpretation and quality of SSEP analysis among different clinicians and hospitals. Conclusions The absence of the SSEP N20 cortical wave remains one of the most reliable early prognostic tools for identifying unfavorable neurologic outcome in the evaluation of patients with severe anoxic-ischemic encephalopathy whether or not they have been treated with TH. When confounding factors are eliminated the false positive rate (FPR) approaches zero.

Bilateral independent periodic lateralised epileptiform discharges at presentation followed by rapid recovery: novel observations from a case of Epstein-Barr virus encephalitis

Bilateral independent periodic lateralised epileptiform discharges (BIPLEDs) in electroencephalograms (EEGs) are commonly caused by anoxic encephalopathy and central nervous system infections. They are associated with coma and high mortality and are thus markers of poor prognosis. We present a case of encephalitis who presented with BIPLEDs in EEG. Though the clinical, EEG and MRI features closely resembled herpes simplex encephalitis, further investigations proved it to be Epstein-Barr virus (EBV) encephalitis. Despite the presence of BIPLEDs in the EEG, the patient had a rapid clinical response to therapy with acyclovir. We emphasise that BIPLEDs may not always indicate poor prognosis especially in the setting of EBV encephalitis.