scholarly journals Clinical presentation and management of proatlas segmentation defect presenting with palatal myoclonus: case report

2015 ◽  
Vol 16 (3) ◽  
pp. 317-321 ◽  
Author(s):  
Raheel Ahmed ◽  
Arnold H. Menezes
Keyword(s):  
Developmental Disorders ◽  
Clinical Presentation ◽  
Signs And Symptoms ◽  
Craniovertebral Junction ◽  
Craniocervical Junction ◽  
Neurological Deficits ◽  
Palatal Myoclonus ◽  
Clinical Presentations ◽  
Neurological Morbidity

Clinical presentation of craniovertebral junction disorders may range from acute catastrophic neurological deficits to insidious signs and symptoms that may mask the underlying etiology. Prompt recognition and treatment is essential to avert long-term neurological morbidity. Proatlas segmentation disorders are a rare group of developmental disorders involving the craniocervical junction. Abnormal bony segmentation leads to malformed bony structures that can in turn lead to neurological deficits through bony compression of the cervicomedullary junction. This report details a proatlas segmentation defect presenting as palatal myoclonus, a rare movement disorder. The clinical presentation, surgical management, and neuroanatomical basis for the disorder is presented. This report highlights the myriad clinical presentations of craniovertebral disorders and emphasizes a rare but treatable etiology for palatal myoclonus.

scholarly journals Variability in Clinical Presentation of Neonatal Stroke: Report of Four Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Sonia Joseph ◽  
Dimitrios Angelis ◽  
Robert Bennett ◽  
Bhargavi Kola ◽  
Amanda Hughes
Keyword(s):  
Risk Factors ◽  
Cognitive Function ◽  
Prenatal Care ◽  
Clinical Presentation ◽  
Neonatal Stroke ◽  
Impaired Cognitive Function ◽  
Neurodevelopmental Disability ◽  
Clinical Presentations

Neonatal stroke can be a cause of long term neurodevelopmental disability, seizures, and impaired cognitive function. We present four cases of neonatal stroke, associated with different risk factors and clinical presentations. Two of these newborns were born to mothers with no prenatal care.

scholarly journals Protracted Bacterial Bronchitis, it is a myth?

2021 ◽  
Vol 20 (4) ◽  
Author(s):  
Ahmad Fadzil
Keyword(s):  
Chronic Cough ◽  
Clinical Presentation ◽  
Signs And Symptoms ◽  
Medical Conditions ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Protracted Bacterial Bronchitis

Protracted wet and productive chronic cough is a symptom that indicate there is an underlying cause and the possibility vary from a simple to serious medical conditions. If the cough is accompanied with specific pointers in signs and symptoms, the diagnosis is apparent. Nevertheless, the challenge is to identify the diagnosis in children who cough without specific pointers. Recently, protracted bacterial bronchitis is one of the conditions that was proposed and postulated to conceive this clinical presentation. Since then, the understanding of PBB has escalated and deepened. However, there is still no unanimous consensus in definition, pathophysiology, diagnostic, treatment, long term outcome and even the entity itself. Further research is required to refine understanding of this condition in several facets therefore patients can be treated accordingly.

scholarly journals The Association Between Delivery of Small-for-Gestational-Age Neonate and Their Risk for Long-Term Neurological Morbidity

2020 ◽  
Vol 9 (10) ◽  
pp. 3199
Author(s):  
Omer Hadar ◽  
Eyal Sheiner ◽  
Tamar Wainstock
Keyword(s):  
Gestational Age ◽  
Small For Gestational Age ◽  
Developmental Disorders ◽  
Proportional Hazards Model ◽  
Survival Curve ◽  
Cohort Analysis ◽  
Cox Proportional Hazards Model ◽  
Increased Risk ◽  
Neurological Morbidity

Small-for-gestational-age (SGA) is defined as a birth weight below the 10th or below the 5th percentile for a specific gestational age and sex. Previous studies have demonstrated an association between SGA neonates and long-term pediatric morbidity. In this research, we aim to evaluate the possible association between small-for-gestational-age (SGA) and long-term pediatric neurological morbidity. A population-based retrospective cohort analysis was performed, comparing the risk of long-term neurological morbidities in SGA and non-SGA newborns delivered between the years 1991 to 2014 at a single regional medical center. The neurological morbidities included hospitalizations as recorded in hospital records. Neurological hospitalization rate was significantly higher in the SGA group (3.7% vs. 3.1%, OR = 1.2, 95% CI 1.1–1.3, p < 0.001). A significant association was noted between neonates born SGA and developmental disorders (0.2% vs. 0.1%, OR = 2.5, 95% CI 1.7–3.8, p < 0.001). The Kaplan-Meier survival curve demonstrated a significantly higher cumulative incidence of neurological morbidity in the SGA group (log-rank p < 0.001). In the Cox proportional hazards model, which controlled for various Confounders, SGA was found to be an independent risk factor for long-term neurological morbidity (adjusted hazard ratio( HR) = 1.18, 95% CI 1.07–1.31, p < 0. 001). In conclusion, we found that SGA newborns are at an increased risk for long-term pediatric neurological morbidity.

Experience with Surgical Decompression of the Arnold-Chiari Malformation in Young Infants with Myelomeningocele

Neurosurgery ◽  
1983 ◽  
Vol 13 (2) ◽  
pp. 147-152 ◽  
Author(s):  
Tae Sung Park ◽  
Harold J. Hoffman ◽  
Bruce E. Hendrick ◽  
Robin P. Humphreys
Keyword(s):  
Chiari Malformation ◽  
Clinical Presentation ◽  
Early Recognition ◽  
Signs And Symptoms ◽  
Complete Recovery ◽  
Neurological Status ◽  
Neurological Deficits ◽  
Arnold Chiari Malformation ◽  
Mild Degree ◽  
Young Infants

Abstract Forty-five infants with myelomeningocele in whom hydrocephalus was absent or adequately controlled developed signs and symptoms of the Arnold-Chiari malformation before the age of 3 months. All of them underwent laminectomy and opening of the dura mater for hindbrain decompression. The clinical presentation included swallowing difficulty, apneic episodes, stridor, bronchial aspiration, arm weakness, and opisthotonos. Within 2 weeks of the initial clinical presentation, the neurological status of 14 patients (31%) deteriorated dramatically and culminated in irreversible neurological deficits. In all patients, compression of the brain stem occurred in the spinal canal. A transverse dural band constricting the dural sac at the C-1 level was noted in 41% of the patients, and a mild degree of arachnoidal adhesion was noted in 23%. The lowermost level of the cerebellar tongue or medullary kink was located at C-1 to C-4 in 28 cases and at C-5 to T-1 in 17 cases. Twenty-eight (62%) of the patients were alive and 17 (38%) had died at the last follow-up assessment. All survivors showed improvement of their overall neurological function. Twenty-four made a complete recovery. The majority of deaths were attributed to respiratory failure. Early recognition of symptoms and prompt decompressive laminectomy are essential for successful management of the Arnold-Chiari malformation in infants.

scholarly journals Combined preoperative traction with instrumented posterior occipitocervical fusion for severe ventral brainstem compression secondary to displaced os odontoideum: technical report of 2 cases

2016 ◽  
Vol 18 (6) ◽  
pp. 724-729 ◽  
Author(s):  
Muhammad M. Abd-El-Barr ◽  
Brian D. Snyder ◽  
John B. Emans ◽  
Mark R. Proctor ◽  
Daniel Hedequist
Keyword(s):  
Craniocervical Junction ◽  
Neurological Function ◽  
Neurological Deficits ◽  
Os Odontoideum ◽  
Surgical Approaches ◽  
Occipitocervical Fusion ◽  
Halo Vest ◽  
Technical Report ◽  
Brainstem Compression

Severe os odontoideum causing ventral brainstem compression is a rare and difficult entity to treat. It is generally accepted that severe os odontoideum causing ventral brainstem compression and neurological deficits warrants surgical treatment. This often requires both anterior and posterior procedures. Anterior approaches to the craniocervical junction are fraught with complications, including infection and risk of injury to neurovascular structures. External traction systems traditionally require long-term bedrest. The authors report 2 cases of severe ventral brainstem compression secondary to displaced os odontoideum and describe their use of extended preoperative halo vest traction to reduce the severe kyphosis and improve neurological function, followed by posterior occipitocervical fusion. Postoperatively both patients showed remarkable improvements in their neurological function and kyphotic deformity. Preoperative halo vest traction combined with posterior occipitocervical fusion appears to be a safe and effective method to treat brainstem compression by severe os odontoideum. It allows for adequate decompression of ventral neural structures and improvement of neurological function, but it is not hindered by the risks of anterior surgical approaches and does not restrict patients to strict bedrest as traditional traction systems. This method of halo vest traction and posterior-only approaches may be transferable to other cervical instability issues with both anterior and posterior pathologies.

Isolated oligohydramnios and long-term neurological morbidity of the offspring

2019 ◽  
Vol 11 (6) ◽  
pp. 648-652
Author(s):  
Avital Dorot ◽  
Tamar Wainstock ◽  
Eyal Sheiner ◽  
Asnat Walfisch ◽  
Daniella Landau ◽  
...  
Keyword(s):  
Developmental Disorders ◽  
Medical Center ◽  
Survival Curve ◽  
Neurological Complications ◽  
Population Based ◽  
Exposed Group ◽  
Kaplan Meier ◽  
Neurological Morbidity ◽  
Independent Association

AbstractThis study aimed to assess the association between maternal-isolated oligohydramnios (IO) and offspring long-term neurological complications. A population-based retrospective cohort study was conducted, including all births at a single tertiary medical center in Israel between the years 1991 and 2014. Multiple pregnancies and potential pregnancy complications associated with oligohydramnios were excluded. The computerized obstetrical database was linked with the computerized dataset of all pediatric hospitalizations of the same medical center. Evaluation of cumulative neurological-associated hospitalizations rate over time was compared using a Kaplan–Meier survival curve. The Weibull survival parametric model was conducted to assess the neurological-associated hospitalization risk in the presence of IO, while accounting for potential confounders. A total of 190,259 pregnancies were included in the study, of which 4063 (2.13%) pregnancies were complicated with IO. Total neurological-related hospitalizations were significantly more common in the IO group (3.7% in the IO group and 3.0% in the comparison group, p = 0.005). Pervasive developmental disorder, movement disorders, developmental disorders, and degenerative and demyelization disorders were all specific neurological diagnoses significantly more common in the exposed group. The survival curve demonstrated a significantly higher cumulative hospitalization rate in the exposed group (log-rank p = 0.001). Using a multivariate model adjusting for gestational age, maternal age, and labor induction, an independent association between IO and long-term neurological morbidity of the offspring was observed (adjusted hazard ratio 1.203; 95% CI 1.02–1.42). In summary, a significant association was found between pregnancies complicated by IO and long-term neurological morbidity of the offspring.

Outcome in neurologically impaired patients with craniovertebral junction tuberculosis: results of combined anteroposterior surgery

2002 ◽  
Vol 97 (2) ◽  
pp. 166-171 ◽  
Author(s):  
Moses Joseph Arunkumar ◽  
Vedantam Rajshekhar
Keyword(s):  
Craniovertebral Junction ◽  
Neurological Deficits ◽  
Retropharyngeal Abscess ◽  
Occipitocervical Fusion ◽  
Limb Weakness ◽  
Long Term Outcome ◽  
Content Type ◽  
Transoral Decompression ◽  
Fine Print

Object. The authors studied the immediate and long-term outcome after transoral decompression, occipitocervical fusion, and antituberculous therapy (ATT) in patients who had neurological deficits due to craniovertebral junction (CVJ) tuberculosis. Methods. In this retrospective study, the authors reviewed the management and outcome in nine consecutive patients in whom features of spinal cord compression were observed and CVJ tuberculosis was diagnosed between 1993 and 1999. They ranged in age from 9 to 55 years. Onset of symptoms was acute or subacute and rapidly progressive (median 4 months, range 1–12 months). Patients presented with neck pain (89%), progressive limb weakness (89%), sensory symptoms (22%), and urinary dysfunction (33%). The mean preoperative functional grade based on the Nurick Scale was 3.4 (range 1–5). The disease caused reducible atlantoaxial dislocation (AAD) in three patients (33%), irreducible AAD in two (22%), basilar impression (BI) in one (11%), AAD with BI in one (11%), and C-2 vertebral body (VB) destruction without dislocation in two (22%). Surgery was performed in all cases. Five patients (56%) underwent transoral odontoidectomy, two (22%) transoral decompression of retropharyngeal abscess and granulation tissue, and two (22%) transoral decompression of abscess and diseased parts of the C-2 VB. All patients then underwent occipitocervical (occiput—C3) fusion in which a contoured Steinmann pin and iliac bone grafts were used. Postoperatively, ATT was prescribed for 18 months. In the immediate postoperative period, function in these patients improved from a mean Nurick grade of 3.4 to 2.3 (p < 0.01). At long-term follow-up examination (median 18.8 months, range 7–46 months) function improved from a mean Nurick grade of 3.4 to 0.3 (range 0–2) (p < 0.001). Conclusions. Patients with CVJ tuberculosis with features of cervical myelopathy are ideally managed with transoral decompressive procedures followed by occipitocervical fusion because this therapy provides immediate neurological improvement, stability, and allows early mobilization. The long-term prognosis in patients with this disease is excellent provided it is treated with appropriate surgical intervention(s) and with adequate duration of ATT.

scholarly journals Neuroinfections: Presentation, Diagnosis, and Treatment of Meningitis and Encephalitis

EMJ Neurology ◽  
2020 ◽  
pp. 93-102
Author(s):  
Kaitlin M. Bowers ◽  
Vishnu V. Mudrakola
Keyword(s):  
Clinical Presentation ◽  
Delayed Diagnosis ◽  
Signs And Symptoms ◽  
Clinical Suspicion ◽  
Diagnosis And Treatment ◽  
Care Providers ◽  
High Clinical Suspicion ◽  
Immunocompromised State ◽  
Neurological Infection

Neuroinfections cause significant morbidity, mortality, and long-term disability. These infections rarely present with the classic signs and symptoms taught in textbooks. Due to the similarities in presentation between neuroinfections and many other disease processes, delayed diagnosis is common. Thus, it is important that care providers have a high clinical suspicion for potential cases because early diagnosis and treatment can significantly improve outcomes. This article serves as a review of the approach to a patient with suspected neurological infection with an emphasis on clinical presentation, diagnosis, and treatment of the major causes of meningitis and encephalitis. Additionally, patients in an immunocompromised state are vulnerable to a whole host of additional neuroinfections that present atypically and will also be addressed.

scholarly journals Erythema Elevatum Diutinum - Two Case Reports, Two Different Clinical Presentations, and a Short Literature Review

2019 ◽  
Vol 7 (18) ◽  
pp. 3039-3042 ◽  
Author(s):  
Uwe Wollina ◽  
Claudia Krönert ◽  
André Koch ◽  
Jacqueline Schönlebe ◽  
Aleksandra Vojvodic ◽  
...  
Keyword(s):  
Literature Review ◽  
Clinical Presentation ◽  
Oral Corticosteroid ◽  
Case Reports ◽  
Response To Treatment ◽  
Clinical Presentations ◽  
Temporary Response ◽  
Erythema Elevatum Diutinum ◽  
Chronic Course

BACKGROUND: Erythema elevatum diutinum (EED) belongs to the spectrum of cutaneous leukocytoclastic vasculitides. EED is a very rare dermatosis presenting with reddish to browning papules and plaques. EED may be associated with infections, hematologic and autoimmune disorders. CASE REPORTS: We present two patients with EED, a 50-year-old woman and a 42-year-old man. While the woman shows an association with colitis ulcerosa, the man had an anti-thrombin deficiency. Treatment was started with oral corticosteroid and dapsone, respectively. In both cases, there was a partial and temporary response. CONCLUSIONS: EED is a rare vasculitis with an unusual clinical presentation and a chronic course. Response to treatment is unsatisfactory and in the long-term run sometimes frustrating.

scholarly journals Correlation between angioarchitectural characteristics of brain arteriovenous malformations and clinical presentation of 183 patients

2021 ◽  
Author(s):  
Ulysses Caus Batista ◽  
Benedito Jamilson Araujo Pereira ◽  
Andrei Fernandes Joaquim ◽  
Helder Tedeschi ◽  
Ronie Leo Piske
Keyword(s):  
Clinical Presentation ◽  
Low Flow ◽  
Neurological Deficits ◽  
Group 4 ◽  
Clinical Presentations ◽  
Group 3 ◽  
Group 5 ◽  
Group 2 ◽  
Presentation Methods ◽  
Group 1

ABSTRACT Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate. Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation. Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental). Results: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01). Conclusions: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

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