Lumbar Puncture Opening Pressure in Patients with Spinal Muscular Atrophy

2021 ◽  
Author(s):  
Lena-Luise Becker ◽  
Claudia Weiß ◽  
Anna Tietze ◽  
Viktoria Martiny ◽  
Angela M. Kaindl
Keyword(s):  
Intracranial Pressure ◽  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Clinical Data ◽  
Side Effect ◽  
Treatment Initiation ◽  
Muscular Atrophy ◽  
Underlying Disease ◽  
Increased Intracranial Pressure ◽  
Opening Pressure

AbstractThe announcement of a hydrocephalus as a possible side effect in patients with spinal muscular atrophy (SMA) receiving the drug nusinersen, promoted major concern and warrants further evaluation. In this retrospective monocentric study, we analyzed clinical data, lumbar puncture opening pressure (LOP) measurement, and ophthalmologic and neuroimaging results in 34 patients with SMA types 1 to 3 undergoing treatment with nusinersen. None of the patients reported symptoms indicative of increased intracranial pressure. In our cohort, the LOP was >20 cm H2O in 25 patients (70.5%), and within this group ≥28 cm H2O in 12 patients (35.3%), in two patients, it was increased prior to treatment initiation. Signs of increased intracranial pressure in ophthalmological assessments or brain imaging were only seen in one patient. We did not identify a correlation between increased LOP and SMA type, scoliosis, or age of the patients; however, it was slightly higher in patients receiving sedation. Our results raise the question whether the LOP is generally increased in SMA as part of the underlying disease, if so, what the etiology is, and whether the increased LOP needs to be treated.

Increased Intracranial Pressure in Patients with Spinal Muscular Atrophy

2019 ◽  
Author(s):  
Lena-Luise Becker ◽  
Anna Tietze ◽  
Claudia Weiß ◽  
Viktoria Martiny ◽  
Angela M. Kaindl
Keyword(s):  
Intracranial Pressure ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Increased Intracranial Pressure

scholarly journals Evaluation of cerebrovascular hemodynamics in patients with idiopathic intracranial hypertension using transcranial Doppler

2020 ◽  
Vol 56 (1) ◽  
Author(s):  
Mahmoud M. Allam ◽  
Hatem A. Almasry ◽  
Sandra M. Ahmed ◽  
Youssuf G. Taha ◽  
Mohammed I. Oraby
Keyword(s):  
Intracranial Pressure ◽  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Transcranial Doppler ◽  
Idiopathic Intracranial Hypertension ◽  
Pulsatility Index ◽  
Research Work ◽  
Increased Intracranial Pressure ◽  
Significant Relation ◽  
Before And After

Abstract Background Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial pressure without a clear cause and can have serious visual effects. Previous research work suggests that transcranial Doppler measurements of pulsatility index correlate accurately with elevated intracranial pressure. Objective To assess the cerebrovascular hemodynamic changes in patients with IIH using transcranial Doppler before and after lumbar puncture and CSF withdrawal. Methods An interventional study conducted on 40 patients (31 females and 9 males) fulfilling the modified Dandy criteria for diagnosis of idiopathic intracranial hypertension, MRI brain, and MRV was done to the patients. Lumbar puncture was done for all included patients to measure intracranial pressure and CSF withdrawal. Transcranial Doppler was performed for all included before and after lumbar puncture and CSF withdrawal and the following parameters were measured: peak systolic velocity (PSV), end diastolic velocity (EDV), resistivity index (RI), and pulsatility index (PI). Results Significant relation was found between grades of papilledema and PSV, RI, and PI (p value 0.012, 0.025, 0.016) but no significant relation was found between grades of papilledema and EDV (0.102). Significant changes occurred in parameters of TCD pre- and post-CSF withdrawal including PSV, EDV, and PI (p value 0.001, 0.015, 0.019) denoting a significant change in cerebral hemodynamics after CSF withdrawal which denotes a decrease in intracranial pressure. Conclusion Increased intracranial pressure significantly affects cerebral blood flow. A normalization of transcranial Doppler parameters occurs following lowering of intracranial pressure through lumbar puncture and CSF withdrawal.

Computed tomography-guided transforaminal lumbar puncture using local anesthesia and a straight 22-gauge spinal needle for intrathecal nusinersen in adults: Findings in 77 procedures

2021 ◽  
pp. 159101992110328
Author(s):  
Mougnyan Cox ◽  
Kofi-Buaku Atsina ◽  
Preethi Ramchand ◽  
Jonathan Ji ◽  
Neda Sedora-Roman ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Local Anesthesia ◽  
Muscular Atrophy ◽  
Intrathecal Administration ◽  
Spinal Needle ◽  
Technical Success ◽  
Computed Tomography Guidance ◽  
Blood Patch

Background Intrathecal nusinersen is the first Food and Drug Administration-approved treatment for spinal muscular atrophy. Reliable intrathecal access is critical for initial and maintenance therapy; however, this can be challenging in older patients with spinal muscular atrophy many of whom have had prior lumbar instrumentation and osseous fusion. Transforaminal lumbar punctures have emerged as a technique for intrathecal access that avoids the hazards of cervical punctures. We describe our technique for transforaminal lumbar punctures under computed tomography guidance using local anesthesia and a straight 22-gauge needle. Methods Following local institutional review board approval, medical records of all patients undergoing computed tomography-guided transforaminal lumbar puncture for intrathecal nusinersen injection were obtained and analyzed. The rate of technical success and immediate complications were recorded. Any delayed complications noted in a 3-day follow-up phone call and future office visit were also recorded. Data collation and analysis were performed using Excel. Results A total of 77 transforaminal lumbar punctures were performed with intrathecal administration of nusinersen, for a 100% technical success rate. Local anesthesia was used in 76 cases, with conscious sedation used in one case. General anesthesia was not used in any case. There were no major complications. One patient had a postdural puncture headache that resolved completely after a transforaminal epidural blood patch performed 4 days later. Conclusions Intrathecal administration of nusinersen is critical for treatment of patients with spinal muscular atrophy. Our described technique allows for reliable access to the intrathecal space using local anesthesia and a straight 22-gauge spinal needle under computed tomography guidance, and is easily reproducible.

Ultrasound‐guided lumbar puncture for nusinersen administration in spinal muscular atrophy patients

2020 ◽  
Author(s):  
D. Veiga‐Canuto ◽  
M. Cifrián‐Pérez ◽  
I. Pitarch‐Castellano ◽  
J.F. Vázquez‐Costa ◽  
F. Aparici
Keyword(s):  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Muscular Atrophy ◽  
Ultrasound Guided

Nusinersen in patients older than 7 months with spinal muscular atrophy type 1

Neurology ◽  
2018 ◽  
Vol 91 (14) ◽  
pp. e1312-e1318 ◽  
Author(s):  
Karolina Aragon-Gawinska ◽  
Andreea M. Seferian ◽  
Aurore Daron ◽  
Elena Gargaun ◽  
Carole Vuillerot ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Function ◽  
Treatment Initiation ◽  
Muscular Atrophy ◽  
Neuromuscular Disorders ◽  
Survival Motor Neuron ◽  
Spinal Muscular Atrophy Type ◽  
Nutritional Data

ObjectiveTo evaluate the safety and clinical efficacy of nusinersen in patients older than 7 months with spinal muscular atrophy type 1 (SMA1).MethodsPatients with SMA1 were treated with nusinersen by intrathecal injections as a part of the Expanded Access Program (EAP; NCT02865109). We evaluated patients before treatment initiation (M0) and at 2 months (M2) and 6 months (M6) after treatment initiation. Survival, respiratory, and nutritional data were collected. Motor function was assessed with the modified Hammersmith Infant Neurologic Examination Part 2 (HINE-2) and physiotherapist scales adjusted to patient age (Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders and the Motor Function Measure 20 or 32).ResultsWe treated 33 children ranging in age from 8.3 to 113.1 months between December 2016 and May 2017. All patients were alive and were continuing treatment at M6. Median progress on the modified HINE-2 score was 1.5 points after 6 months of treatment (p < 0.001). The need for respiratory support significantly increased over time. There were no statistically significant differences between patients presenting with 2 and those presenting with 3 copies of the survival motor neuron 2 (SMN2) gene.ConclusionsOur results are in line with the phase 3 study for nusinersen in patients with SMA1 treated before 7 months of age and indicate that patients benefit from nusinersen even at a later stage of the disease.ClinicalTrials.gov identifier:NCT02865109.Classification of evidenceThis study provides Class IV evidence that for patients with SMA1 who are older than 7 months, nusinersen is beneficial.

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