Movement Disorder as Unusual Manifestation of Chiari Malformation Type II in a Newborn

2020 ◽  
Author(s):  
Lara Mastino ◽  
Roberto Mai ◽  
Marco Cenzato ◽  
Giuseppe D'Aliberti ◽  
Giuseppe Talamonti
Keyword(s):  
Head And Neck ◽  
Movement Disorder ◽  
Ventriculoperitoneal Shunt ◽  
Movement Disorders ◽  
Chiari Malformation ◽  
Ventricular Dilatation ◽  
Type Ii ◽  
Unusual Manifestation ◽  
Myelomeningocele Repair

AbstractChiari malformation type II (CM-II) is a hindbrain developmental malformation. Movement disorders are rarely described as associated with this condition. We describe the case of a newborn affected by CM-II presenting with head and neck tremors a few days after myelomeningocele repair surgery. Later, self-resolving episodes of expiratory apneas arose. Cranial ultrasounds showed progressive ventricular dilatation. She underwent ventriculoperitoneal shunt followed by craniocervical bony decompression. After surgery, both apnea and tremors disappeared. This particular presentation of CM-II is very rare and potentially difficult to diagnose.

Postnatal ascent of the cerebellar tonsils in Chiari malformation Type II following surgical repair of myelomeningocele

2008 ◽  
Vol 2 (3) ◽  
pp. 188-193 ◽  
Author(s):  
Nobuhito Morota ◽  
Satoshi Ihara
Keyword(s):  
Chiari Malformation ◽  
Surgical Repair ◽  
Type Ii ◽  
Spinal Level ◽  
Vp Shunt ◽  
Group 2 ◽  
Myelomeningocele Repair ◽  
Cerebellar Tonsils ◽  
Group 1

Object Postnatal improvement in Chiari malformation type II (CM-II) following surgical repair of myelomeningocele was evaluated. Methods The authors reviewed data obtained in 20 cases in which patients underwent postnatal myelomeningocele repair within the first 48 hours after birth between October 2002 and September 2006. In 14 patients (Group 1), myelomeningocele was diagnosed in utero and the infants were delivered by cesarean section at 35–39 weeks' gestation (mean 36.4). The 6 infants in Group 2 were born after full-term gestation (39–41 weeks), and their myelomeningoceles were diagnosed postnatally. In all 20 patients, the myelomeningoceles were surgically repaired postnatally. Dynamic change of the herniated cerebellar tonsils in CM-II before and after the myelomeningocele repair, associated hydrocephalus, and symptomatic CM-II were analyzed. Results In Group 1, the CM-II was confirmed before myelomeningocele repair in 13 cases (93%). The spinal level of the caudal end of the cerebellar tonsils ranged from C-2 to C-7. Ascent of the cerebellar tonsils was observed in 11 patients (range 1–4 spinal levels, mean 2 levels) and continued even after ventriculoperitoneal (VP) shunt placement in most patients. A VP shunt was required for the treatment of hydrocephalus in 12 patients (86%). Symptomatic CM-II developed in 8 of 13 patients (61%), 3 of whom required surgical decompression. In Group 2, CM-II was confirmed in 5 infants (83%), with the cerebellar tonsils at a spinal level of C-2 to C-4 or C-5. Ascent of the cerebellar tonsils was observed in 4 patients (range 1–1.5 spinal levels, average 1.1 levels), and no patient had symptomatic CM-II. A VP shunt was placed in 5 patients (83%). No patient was lost to follow-up during the 18-month follow-up period. The only statistically significant difference between the 2 groups was the presence of symptomatic CM-II in Group 2 (p = 0.02). Conclusions Patients showed ascent of the cerebellar tonsils after postnatal myelomeningocele repair. Placement of a VP shunt helped promote the ascent. However, postnatal myelomeningocele repair in the patients in Group 1 failed to consistently prevent development of symptomatic CM-II. This limited experience suggests that postnatal repair of myelomeningocele can partially reverse the anatomical CM-II, but symptomatic CM-II cannot be prevented in some patients when the repair is performed after 36 weeks' gestation.

Open fetal surgery for myelomeningocele

2012 ◽  
Vol 9 (3) ◽  
pp. 265-273 ◽  
Author(s):  
Nalin Gupta ◽  
Jody A. Farrell ◽  
Larry Rand ◽  
Charles B. Cauldwell ◽  
Diana Farmer
Keyword(s):  
Clinical Trial ◽  
Informed Consent ◽  
Ventriculoperitoneal Shunt ◽  
Chiari Malformation ◽  
Treatment Plan ◽  
Fetal Surgery ◽  
Treatment Decision ◽  
Type Ii ◽  
Final Treatment ◽  
Fetal Repair

In a recently completed randomized, prospective clinical trial, fetal repair for myelomeningocele was shown to result in reduced rates of hydrocephalus requiring placement of a ventriculoperitoneal shunt, improvement in Chiari malformation Type II, and improvement in neurological function compared with standard postnatal repair. Successful fetal surgery requires the active participation and interaction of several clinical teams. Each group has a specific role, and overlap is often required at different points of the treatment plan. Extensive multispecialty discussions with the patient and family are necessary before informed consent can be obtained. Fetal surgery carries significant risks to the mother and fetus and these must be carefully considered prior to a final treatment decision. This review will summarize the evaluation and treatment of patients undergoing fetal repair for myelomeningocele at one institution.

Meconium staining of the brainstem with open myelomeningocele

2013 ◽  
Vol 11 (2) ◽  
pp. 150-153 ◽  
Author(s):  
Sandi Lam ◽  
Ramesh Grandhi ◽  
Stephanie Greene
Keyword(s):  
Chiari Malformation ◽  
Perinatal Care ◽  
Type Ii ◽  
Meconium Staining

Meconium staining of open myelomeningoceles has been reported to occur both prenatally and postnatally, but meconium staining of the brainstem has not been previously documented. The authors present a case of meconium staining of the brainstem in an infant with a meconium-stained myelomeningocele, Chiari malformation Type II, and hydrocephalus and discuss possible implications for prenatal and perinatal care.

Gamma knife radiosurgery as a lesioning technique in movement disorder surgery

1997 ◽  
Vol 2 (3) ◽  
pp. E13 ◽  
Author(s):  
Ronald F. Young ◽  
Anne Shumway-Cook ◽  
Sandra S. Vermeulen ◽  
Peter Grimm ◽  
John Blasko ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Movement Disorder ◽  
Gamma Knife ◽  
Movement Disorders ◽  
Rating Scale ◽  
Gamma Knife Radiosurgery ◽  
Magnetic Resonance Images ◽  
Maximum Deviation

Fifty-five patients underwent radiosurgical placement of lesions either in the thalamus (27 patients) or globus pallidus (28 patients) for treatment of movement disorders. Patients were evaluated pre- and postoperatively by a team of observers skilled in the assessment of gait and movement disorders who were blinded to the procedure performed. They were not associated with the surgical team and concomitantly and blindly also assessed a group of 11 control patients with Parkinson's disease who did not undergo any surgical procedures. All stereotactic lesions were made with the Leksell gamma unit using the 4-mm secondary collimator helmet and a single isocenter with dose maximums from 120 to 160 Gy. Clinical follow-up evaluation indicated that 88% of patients who underwent thalamotomy became tremor free or nearly tremor free. Statistically significant improvements in performance were noted in the independent assessments of Unified Parkinson's Disease Rating Scale (UPDRS) scores in the patients undergoing thalamotomy. Eighty-five and seven-tenths percent of patients undergoing pallidotomy who had exhibited levodopa-induced dyskinesias had total or near-total relief of that symptom. Clinical assessment indicated improvement of bradykinesia and rigidity in 64.3% of patients who underwent pallidotomy. Independent blinded assessments did not reveal statistically significant improvements in Hoehn and Yahr scores or UPDRS scores. On the other hand, 64.7% of patients showed improvements in subscores of the UPDRS, including activities of daily living (58%), total contralateral score (58%), and contralateral motor scores (47%). Ipsilateral total UPDRS and ipsilateral motor scores were both improved in 59% of patients. One (1.8%) of 55 patients experienced a homonymous hemianopsia 9 months after pallidotomy due to an unexpectedly large lesion. No other complications of any kind were seen. Follow-up neuroimaging confirmed correct lesion location in all patients, with a mean maximum deviation from the planned target of 1 mm in the vertical axis. Measurements of lesions at regular interals on postoperative magnetic resonance images demonstrated considerable variability in lesion volumes. The safety and efficacy of functional lesions made with the gamma knife appear to be similar to those made with the assistance of electrophysiological guidance with open functional stereotactic procedures. Functional lesions may be made safely and accurately using gamma knife radiosurgical techniques. The efficacy is equivalent to that reported for open techniques that use radiofrequency lesioning methods with electrophysiological guidance. Complications are very infrequent with the radiosurgical method. The use of functional radiosurgical lesioning to treat movement disorders is particularly attractive in older patients and those with major systemic diseases or coagulopathies; its use in the general movement disorder population seems reasonable as well.

scholarly journals Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type II

2009 ◽  
Vol 67 (1) ◽  
pp. 29-34 ◽  
Author(s):  
Paulo Sérgio Azeredo Henriques Filho ◽  
Riccardo Pratesi
Keyword(s):  
Sleep Apnea ◽  
Sleep Disorders ◽  
Attention Deficit ◽  
Chiari Malformation ◽  
Sleep Apnea Syndrome ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Type Ii ◽  
Sleep Behavior ◽  
Apnea Syndrome

BACKGROUND: Attention deficit may be related to sleep disorders in Chiari malformation type II (CMII). Our aim is identify sleep disorders and their specific contribution in attention deficit. METHOD: We selected 24 patients with CM II and 24 without CM II. DSM-IV criteria and a neuropsychological analysis were applied in all. All patients underwent full night polysomnography. RESULTS: 14 CM II patients presented sleep apnea syndrome, REM sleep behavior disorder and periodic limb movement in sleep; six patients without CM II presented sleep apnea syndrome. Among these patients, 12 (six with CM II and six without CM II) presented attention deficit related to the sleep disorders. CONCLUSION: Sleep disorders may impair cognitive functions, as attention, and contribute to poor quality of learning also in patients with CM II.

Morphometric analysis of posterior fossa after in utero myelomeningocele repair

2011 ◽  
Vol 7 (4) ◽  
pp. 362-368 ◽  
Author(s):  
Ryan A. Grant ◽  
Gregory G. Heuer ◽  
Geneive M. Carrión ◽  
N. Scott Adzick ◽  
Erin S. Schwartz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Surgical Repair ◽  
Correlation Coefficients ◽  
In Utero ◽  
Clinical Effects ◽  
Type Ii ◽  
Tonsillar Herniation ◽  
Myelomeningocele Repair ◽  
Repair Group

Object Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF). Methods The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients. Results Initially, the in utero CSO angle was significantly more acute in all patients with MMC—prenatally and postnatally repaired—as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = −0.58) and in the antenatal repair group (R = −0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015). Conclusions Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.

Classification and Approach to Movement Disorders

2021 ◽  
pp. 571-575
Author(s):  
Paul E. Youssef ◽  
Kenneth J. Mack ◽  
Kelly D. Flemming
Keyword(s):  
Motor Activity ◽  
Movement Disorder ◽  
Movement Disorders ◽  
Normal Flow ◽  
Slow Movement ◽  
Involuntary Movements ◽  
P Movement

Movement disorders are conventionally divided into 2 major categories. Hyperkinetic movement disorders (also called dyskinesias) are excessive, often repetitive, involuntary movements that intrude into the normal flow of motor activity. This category includes chorea, dystonia, myoclonus, stereotypies, tics, and tremor. Hypokinetic movement disorders are akinesia (lack of movement), hypokinesia (reduced amplitude of movement), bradykinesia (slow movement), and rigidity. Parkinsonism is the most common hypokinetic movement disorder. In childhood, hyperkinetic disorders are common, whereas hypokinetic movement disorders are relatively uncommon.

Sleep-Related Movement Disorders

2020 ◽  
pp. 193-214
Author(s):  
Ravi Gupta
Keyword(s):  
Movement Disorder ◽  
Psychiatric Disorders ◽  
Iron Metabolism ◽  
Restless Legs Syndrome ◽  
Movement Disorders ◽  
Genetic Factors ◽  
Psychotropic Medications ◽  
Predisposing Factors ◽  
Treatable Condition

Sleep-related movement disorders include disorders that manifest as simple and mostly stereotyped movements occurring at sleep–wake interface or during sleep. Restless legs syndrome (RLS), the most common sleep-related movement disorder. RLS is a treatable condition that psychiatrists must be familiar with due to its common comorbidity with a broad range of psychiatric disorders. In addition, commonly used psychotropic medications are known to induce or worsen RLS symptoms in predisposed individuals, and these symptoms may be mistaken for akathisia or sleep-initiation insomnia and lead to mismanagement. This chapter discusses, among other RLS-related topics, the diagnosis, epidemiology, predisposing factors, genetic factors, and the role of iron metabolism.

Hypokinetic Movement Disorders: Parkinson Disease

2021 ◽  
pp. 576-582
Author(s):  
Sarah M. Tisel ◽  
Bryan T. Klassen
Keyword(s):  
Cell Death ◽  
Substantia Nigra ◽  
Parkinson Disease ◽  
Movement Disorder ◽  
Movement Disorders ◽  
Dopaminergic Neurons ◽  
Microscopic Level ◽  
Rest Tremor ◽  
Macroscopic Level ◽  
Progressive Degeneration

Parkinson disease (PD) is the classic hypokinetic movement disorder and one of the most common and widely recognized neurodegenerative conditions. PD is distinct from parkinsonism, a term that refers to a syndrome of rest tremor, bradykinesia, rigidity, and postural instability. The mechanism behind the progressive degeneration and cell death that result in PD is not precisely understood. Substantia nigra depigmentation occurs on a macroscopic level and loss of dopaminergic neurons and gliosis on a microscopic level.

Sign in / Sign up

Export Citation Format

Share Document