Greater occipital nerve blockade using ultrasound guidance for the headache of spontaneous intracranial hypotension - A case report -

BackgroundSpontaneous intracranial hypotension occurs due to cerebrospinal fluid leakage from the spinal column, and orthostatic headache is the most common clinical presentation. Recent studies showed that bilateral greater occipital nerve blockade demonstrated clinical efficacy in relieving post-dural puncture headache after caesarean section. CaseA 40-year-old male who presented severe orthostatic headache was consulted to our pain clinic from neurology department. He initially felt a dull nature pain over the whole occipital area which then spread over the frontal and parietal areas. His headache was combined with nausea and vomiting. An epidural blood patch was delayed until final cisternography, and bilateral greater occipital nerve blockade using ultrasound guidance was performed instead. After the blockade, the previously existing headache around the occipital and parietal areas disappeared completely, but mild headache persisted around the frontal area.ConclusionsGreater occipital nerve blockade could be a good therapeutic alternative to improve headache resulting from spontaneous intracranial hypotension.

Update on “Treatment of orthostatic headache without intracranial hypotension: A case report” – nine years later

Background In 2013, one of the authors described a 36-year-old female with orthostatic headache without documented intracranial hypotension or evidence of cerebrospinal fluid leak, despite extensive workup. Headache was unresponsive to conservative treatment since 2010, showed only transient benefit after repeated epidural blood patches while vitamin A supplementation resulted in progressive improvement. Case Since 2013, the patient followed a relapsing and remitting course yet relapse control became difficult after a drug induced liver injury required vitamin A discontinuation in 2017, when her headache became chronic. Greater occipital nerve blocks provided pain relief as alternative but were stopped due to the pandemic and her latest severe relapse, in late 2020, required not only restarting anaesthetic blocks and aggressive medication management, but also reassessing and treating comorbidities (obstructive sleep apnoea and major depressive disorder) with modest benefit. Conclusion Orthostatic headache without intracranial hypotension is rare, with only 28 cases reported so far, all treated empirically and all treatment options revealing to be mostly ineffective. Vitamin A anecdotally appeared to be useful in our case but had to be stopped for severe side effects, so unfavourable long-term prognosis, in ours and 2/3 of the reported cases, seems to be the rule in this intriguing entity.

Iatrogenic Intracranial Hypotension after Surgery of Adolescent Idiopathic Scoliosis due to a Misplaced Screw within the Thoracic Thecal Sac

We present the case of a postoperative intracranial hypotension due to a misplaced intrathecal screw. Although typical symptoms such as orthostatic headache were present immediately after surgery, diagnosis was hidden until 20 years later, resulting in a disastrous clinical course.

A spontán intracranialis hypotensio diagnosztikája mágneses rezonanciás képalkotással

Összefoglaló. A klasszikus esetben ortosztatikus fejfájást okozó, spontán intracranialis hypotensiót az esetek túlnyomó többségében a gerinccsatornában, annak nyaki-háti átmenetében, illetve a háti szakaszán található liquorszivárgás okozza. Meglévő kötőszöveti betegség, degeneratív gerincbetegségek, illetve kisebb traumák szerepet játszhatnak a szivárgás kialakulásában. Az ortosztatikus fejfájás létrejöttében szerepet játszhat a meningealis szerkezetek, érzőidegek és hídvénák vongálódása. A klasszikus pozicionális, ortosztatikus fejfájásban szenvedő betegek körében gondolni kell a spontán intracranialis hypotensio lehetőségére, és az agykoponya, illetve a gerinc kontrasztanyaggal végzett mágneses rezonanciás vizsgálata (MRI) javasolt. A kontrasztanyaggal végzett koponya-MRI-vel klasszikus esetben diffúz, nem nodularis, intenzív, vaskos pachymeningealis kontrasztanyag-halmozás, kitágult vénássinus-rendszer, subduralis effusiók és az agytörzs caudalis diszlokációja („slumping”) látható. Fontos azonban szem előtt tartani, hogy az esetek 20%-ában ezen eltérések nem detektálhatók. Jó minőségű, randomizált, kontrollált vizsgálatok nem történtek, a kezelés hagyományokon alapul. Kezdetben általában konzervatív terápiát alkalmaznak (ágynyugalom, koffein- és folyadékbevitel), ennek hatástalansága esetén epiduralis sajátvér-injekció, epiduralis fibrinragasztó-injektálás, illetve sebészi terápia jöhet szóba. Orv Hetil. 2021; 162(7): 246–251. Summary. Spontaneous intracranial hypotension, the classic feature of which is orthostatic headache, is most commonly caused by a cerebrospinal fluid leakage at the level of the spinal canal, in most cases at the thoracic level or cervicothoracic junction. Underlying connective tissue disorders, minor trauma, degenerative spinal diseases may play a role in the development of cerebrospinal fluid leaks. Traction on pain-sensitive intracranial and meningeal structures, particularly sensory nerves and bridging veins, may play a role in the development of orthostatic headache. In the case of patients with classic orthostatic headache, the possibility of spontaneous intracranial hypotension should be considered, and if suspected, brain magnetic resonance imaging (MRI) with gadolinium and additional spine MRI are recommended. Diffuse, non-nodular, intense, thick dural enhancement, subdural effusions, engorgement of cerebral venous sinuses, sagging of the brain are typical features on brain MRI, which, however, remain normal in up to 20 percent of patients with spontaneous intracranial hypotension. Unfortunately, no randomized clinical trials have evaluated the effectiveness of the various treatment strategies and no definitive treatment protocols have been established. In clinical practice, the first-line treatment of spontaneous intracranial hypotension is conservative (bed rest, caffeine and fluid intake). If conservative therapy is not effective, epidural blood patch, epidural fibrin glue, or surgical repair should be considered. Orv Hetil. 2021; 162(7): 246–251.

The importance of cephaliatric anamnesis in the diagnosis and early treatment of spontaneous intracranial hypotension: an experience told in a case report

Introduction: Spontaneous intracranial hypotension (SIH) is a rare syndrome, still underdiagnosed. It manifests with orthostatic headache and improves with decubitus, and may present nausea, vomiting, diplopia, vestibular and auditory symptoms. The main cause of SIH is spontaneous CSF leaks usually in the cervicothoracic transition. Case report: RSA, male, 36 years, he presented holocranial headache triggered in the orthostatic position and decubitus improvement associated with nausea. He denied visual complaints, fever, trauma or invasive procedures. Neurological examination: discrete neck stiffness, fundoscopy and others exams without abnormalities. Brain and cervical spine MRI, also venous AngioMRI were normal. Dorsal spine MRI with extradural collection. Arterial AngioMRI with 2,50x2,0mm aneurysmatic dilation in the right supraclinoid internal carotid artery. Opening pressure of CSF 6cmH2 O. Analgesia, decubitus rest and parsimonious hydration were performed. In cisternoscintigraphy, CSF leakage into the extradural space at the level of D3/D4 and D4/D5 on the left, delay in the rise of the tracer for brain convexities in 24 hours images, suggestive of CSF hypotension. Blood patch guided by radioscopy was performed, with improvement after 2 weeks of the 3rd procedure. Conclusion: Knowing the types of headache and its etiologies is essential to orientate diagnosis and treatment, avoiding unnecessary exams. In this case report, the microaneurysm found did not justify the complaint of orthostatic headache. The rapid diagnosis of spontaneous CSF leaks provided early treatment avoiding complications such as subdural hematomas, cerebral venous thrombosis, pituitary dysfunction.

Orthostatic Headache in Children Including Postural Tachycardia Syndrome and Orthostatic Hypotension: A Near-Infrared Spectroscopy Study

Background and aim: Although head and/or neck pain attributed to orthostatic hypotension is included in international guidelines, its mechanisms and relevance remain unknown. This study examined the term’s relevance and aimed to elucidate the associated clinical features. Methods: An active stand test was performed to evaluate fluctuations in systemic and cerebral circulation in children and adolescents reporting complaints in the absence of a confirmed organic disorder. The subjects were categorized based on orthostatic headache presence/absence, and their characteristics and test results were compared. Results: Postural tachycardia syndrome was observed in 50.0% of children with, and 55.1% without, orthostatic headache. For orthostatic hypotension, the respective values were 31.3% and 30.6%. A history of migraine was more prevalent in children with orthostatic headaches (64.1% vs. 28.6%; p < 0.01). The observed decrease in the cerebral oxygenated hemoglobin level was larger in children with orthostatic headaches (Left: 6.3 (3.2–9.4) vs. 4.1 (0.8–6.1); p < 0.01, Right: 5.3 (3.1–8.6) vs. 4.0 (0.8–5.9); p < 0.01). Conclusion: Fluctuations in cerebral blood flow were associated with orthostatic headaches in children, suggesting that the headaches are due to impaired intracranial homeostasis. As orthostatic headache can have multiple causes, the term “head and/or neck pain attributed to orthostatic (postural) hypotension” should be replaced with a more inclusive term.

Spontaneous intracranial hypotension combined with subarachnoid hemorrhage: report of 2 cases

Background: Spontaneous intracranial hypotension (SIH) combined with subarachnoid hemorrhage (SAH) has rarely been reported. Herein, we report two patients with SIH who suffered from diffuse non-aneurysmal SAH and expanded the symptom spectrum of SIH.Case report: ① A 55-year-old male was diagnosed with SIH based on orthostatic headache and diffuse pachymeningeal enhancement on brain MRI. One more month later, his headache was exacerbated, and brain CT showed diffuse SAH. Lumber puncture showed bloody CSF with a low CSF pressure of 20 mmH2O after a 30 ml intrathecal injection of saline. The patient was treated with a lumbar epidural blood patch and recovered. ② A 41-year-old male presented with orthostatic headache and nuchal pain. The brain CT scan confirmed the diagnosis of SAH. Brain MRI revealed diffuse dural thickening and bilateral frontoparietal subdural fluid collection. Lumber puncture showed bloody CSF with low CSF pressure. Then, an epidural blood patch was performed with satisfactory results.Conclusion: Dilation and rupture of intracranial venous structures might play significant roles in SIH combined with SAH. We should be alert to SIH patients who develop a new persistent severe headache without relief after lying down or a suddenly changed state of consciousness.

Lumbar epidural blood patch: effectiveness on orthostatic headache and MRI predictive factors in 101 consecutive patients affected by spontaneous intracranial hypotension

OBJECTIVEAlthough epidural blood patch (EBP) is considered the gold-standard treatment for drug-resistant orthostatic headache in spontaneous intracranial hypotension (SIH), no clear evidence exists regarding the best administration method of this technique (blind vs target procedures). The aim of this study was to assess the long-term efficacy of blind lumbar EBP and predictors on preoperative MRI of good outcome.METHODSLumbar EBP was performed by injecting 10 ml of autologous venous blood, fibrin glue, and contrast medium in 101 consecutive patients affected by SIH and orthostatic headache. Visual analog scale (VAS) scores for headache were recorded preoperatively, at 48 hours and 6 months after the procedure, and by telephone interview in July 2017. Patients were defined as good responders if a VAS score reduction of at least 50% was achieved within 48 hours of the procedure and lasted for at least 6 months. Finally, common radiological SIH findings were correlated with clinical outcomes.RESULTSThe median follow-up was 60 months (range 8–135 months); 140 lumbar EBPs were performed without complications. The baseline VAS score was 8.7 ± 1.3, while the mean VAS score after the first EBP procedure was 3.5 ± 2.2 (p < 0.001). The overall response rate at the 6-month follow-up was 68.3% (mean VAS score 2.5 ± 2.4, p < 0.001). Symptoms recurred in 32 patients (31.7%). These patients underwent a second procedure, with a response rate at the 6-month follow-up of 78.1%. Seven patients (6.9%) did not improve after a third procedure and remained symptomatic. The overall response rate at the last follow-up was 89.1% with a mean VAS score of 2.7 ± 2.3 (p < 0.001). The only MRI predictors of good outcome were location of the iter > 2 mm below the incisural line (p < 0.05) and a pontomesencephalic angle (PMA) < 40° (p < 0.05).CONCLUSIONSLumbar EBP may be considered safe and effective in cases of drug-refractory SIH. The presence of a preprocedural PMA < 40° and location of the iter > 2 mm below the incisural line were the most significant predictors of good outcome. Randomized prospective clinical trials comparing lumbar with targeted EBP are warranted to validate these results.

Fast effective treatment of spontaenous CSF leak causing intracranial hypotension and debilitating orthostatic headache

We are reporting a case series describing clinical, laboratory, MRI and CT myelogram imaging and treatment results for 4 patients with spontaneous cerebral spinal fluid (CSF) leak with subsequent intracranial hypotension; all of which resolved via blood patches. A 33-year-old woman (case 1) presented abrupt onset severe pain in the back and head; without preceding trauma or procedure. MRI brain revealed leptomeningeal enhancement, after CT myelogram identifying a CSF leak she received a 20ml epidural blood patch in the lumbar area. By the next day her symptoms had completely resolved. A 36year old woman (case 2) with 1 year-long history of positional headaches. Her CT Myelogram showed frequent multilevel Tarlov Cysts; one of which displayed leakage. Her orthostatic headaches disappeared after repeated EBP. A middle aged male (case 3) had an acute onset headache which persisted with orthostatic features since 6 weeks. He had complete remission after one epidural patching. A 47 year old man (case 4) presented with 5 days of typical orthostatic headaches, CT Myelogramm demonstrated a leaking thoracolumbar cyst. He required 2 EBP within 3 days to completely recover. Our cases support the notion that blood patching is a fast, effective and safe treatment for an increasingly recognized diagnosis of orthostatic headache caused by spontaneous intracranial hypotension.

Headache due to spontaneous spinal cerebrospinal fluid leak secondary to cerebrospinal fluid-venous fistula: Case series

Objective Cerebrospinal fluid-venous fistula is an uncommon cause of spontaneous spinal cerebrospinal fluid leak (SSCSFL). We aim to describe the clinical presentation, imaging evaluation, treatment and outcome of SSCSFL secondary to cerebrospinal fluid-venous fistula. Methods A retrospective review was undertaken of SSCSFL cases secondary to cerebrospinal fluid-venous fistula confirmed radiologically or intraoperatively, seen at our institution from January 1994 to March 2019. Cases with undetermined SSCSFL etiology, alternative etiology or unconfirmed fistula were excluded. Results Forty-four of 156 patients met the inclusion criteria (31 women, 13 men). Mean age of symptom onset was 52.6 years (SD 8.7, range 33–71 years). Headache was the presenting symptom in almost all, typically daily (69%), and most often in occipital/suboccipital regions. Headache character was most commonly pressure (38%), followed by throbbing/pulsing (21.4%). Orthostatic headache worsening occurred in 69% and an even greater percentage of patients (88%) reported Valsalva-induced headache exacerbation or precipitation. Headache occurred in isolation to Valsalva maneuvers in 12%. Of 37 patients with documented cerebrospinal fluid opening pressure, 13% were <6 cmH2O; 84%, 7–20 cmH2O; and one, 25 cmH2O. Fistulas were almost exclusively thoracic (95.5%). Only one patient responded definitively to epidural blood patch (EBP). Forty-two patients underwent surgery. Most improved following surgery; 48.7% were completely headache free and 26.8% had at least 50% improvement. Conclusion In our series, cerebrospinal fluid-venous fistula was associated with a greater occurrence of Valsalva-induced headache exacerbation or precipitation than orthostatic headache and did not respond to EBP. Surgery provided significant improvement. Cerebrospinal fluid-venous fistula should be considered early in the differential diagnosis of Valsalva-induced (“cough”) headache.