Table 1
Summary of publications on SIH/BSS that have reported gait dysfunction as a predominant symptom.
| AUTHOR, YEAR | AGE/SEX | TYPICAL SIH SYMPTOMS NOTED? | TYPICAL FBSS SYMPTOMS NOTED? | OTHER SYMPTOMS | TYPE OF GAIT ABNORMALITY | KEY EXAMINATION FINDING | IMAGING ABNORMALITIES | TREATMENT | IMPROVEMENT OF SIH/FBSS SYMPTOMS? | IMPROVEMENT OF MOVEMENT DISORDERS? | FOLLOW UP BRAIN IMAGING |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Nowak et al. 2003 | 50/M | No | no | Visual blurring | Paroxysmal episodes of severe unsteadiness when standing and walking | Not mentioned apart from moderate unsteadiness | B/L subdural hygromas. CSF leak at T12–L1levels | EBP | – | Complete resolution | Not done |
| Peng et al. 2004 | 50/F | Yes (orthostatic headache, nausea, vomiting) | Yes (short term memory loss) | Sensorineural deafness | Gait ataxia | Impaired tandem gait, MMSE 26/30, positive Babinski signs | Brain sagging, B/L subdural collection, diffuse dural enhancement | EBP | Headaches resolved | Improved significantly, not mentioned if it fully resolved. | Not done |
| Weisfelt et. al. 2004 | 51/M | Yes (6 weeks of headaches) | Yes (1 week cognitive decline and fluctuating consciousness) | Somnolence and apneas | Gait ataxia (details not available) | Fluctuating consciousness level, positive Babinski sign | B/L subdural effusions, pachymeningeal enhancement, descent of cerebellar tonsil, flattening of pons | EBP and lumbar infusion of normal saline | Headache resolved | All neurological symptoms reportedly resolved | Restoration of cerebral descent |
| Mistry et al. | 39/M | Yes (4 months of orthostatic headache, 4 weeks of nausea) | Yes (4 weeks of mood/personality changes) | Diplopia | Gait ataxia (details not available) | No focal neurological signs | Downward displacement of diencephalon, B/L subdural collections | EBP (failed), targeted blood patch (repeated 2 times) | Resolved temporarily on first targeted blood patch before returning | Significantly improved only after 3 targeted blood patches | Not done |
| Uysal et al. 2008 | 5/F | Yes (1 month of orthostatic headaches) | no | Sudden onset hearing ess | Gait ataxia (details not available) | Ataxic gait, hearing loss | Enlargement of cervical venous plexus, dural leak at level of L3–L4 vertebraepk | Oral caffeine, EBP × 2 | Complete resolution after EBP | Improvement with oral caffeine, Complete resolution with EBP × 2 | Not done |
| Devine et al. 2009 | 46/M | Yes (headache, neck pain, aural fullness) | Yes (memory disturbance) | Gait unsteadiness | Impaired tandem gait, dysmetria on finger-nose-finger, postural tremor of arms, MMSE 20/30 | Brainstem sagging, distortion of midbrain, B/L transtentorial herniation, B/L subdural collections | Bed rest in trendelenburg, IV caffeine, oral dexamethasone | Excellent response | Excellent response | Not done | |
| Sasikumar et al. 2018 | 64/M | No | no | Broad based stance sway fluctuations, narrow stride length, decrease stride velocity | None other gait abnormality for both cases | Low-lying cerebellar tonsils, diffuse pachymeningeal thickening, subdural effusions | Multiple non-targeted EBP. | Not mentioned | “Remarkable improvements” on quantitative gait analysis 1 week after EBP. | MRI did not show radiologic eveidence of SIH | |
| 80/F | No | No | Details are not mentioned except “side to side” while walking | Venous distention, pachymeningeal thickening and subdural effusions. | Declined treatment | Spontaneous resolution of symptoms | |||||
| Domínguez et al. 2023 | 53/M | Yes (6 months of orthostatic headache and tinnitus) | Yes (2 months of behavioral changes, delusional ideation) | Recurrent falls, gait ataxia with retropulsion | Dysmetria in all limbs, Kinetic tremor of arms, Severe cognitive dysfunction tests | Descent of cerebellar tonsils, transtentorial herniation, distortion of brainstem structures and descent of splenium of corpus callosum, CSF leak at D5 level. | EBP × 2 | Sustained recovery of cognitive symptoms | Complete and sustained recovery after EBP (SARA score improved from 16 to 0). | Resolution of brain sagging |
[i] BSS: Brain sagging syndrome, CSF: Cerebrospinal fluid, MMSE: Mini Mental Status Examination, SARA: Scale for the assessment and rating of ataxia (SARA), SIH: Spontaneous intracranial hypotension, EBP: Epidural blood patch.
Table 2
Summary of publications on SIH/BSS that have reported tremor as a predominant symptom.
| AUTHOR, YEAR | AGE/SEX | TYPICAL SIH SYMPTOMS NOTED? | TYPICAL FBSS SYMPTOMS NOTED? | OTHER SYMPTOMS | CHARACTERISTICS OF TREMOR | KEY EXAMINATION FINDING | IMAGING ABNORMALITIES | TREATMENT | IMPROVEMENT OF SIH/FBSS SYMPTOMS? | IMPROVEMENT OF MOVEMENT DISORDERS? | FOLLOW UP BRAIN IMAGING |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Turgut et al. 2009 | 57/M | No | No | none | B/L R > L 7 Hz postural tremor with intention component | Tremor absent on rest, not associated with bradykinesia, rigidity, dystonia. | B/L pachymeningeal enhancement, brain sagging, CSF leak at left thoraco-lumbar area due to a ruptured meningeal diverticula | Epidural blood patch at thoracolumbar junction | – | Complete resolution of tremor at 2 months after EBP | Decrease in meningeal thickening and resolution of brain sagging |
| Mokri et al. 2014 | 51/W (patient 2) | Yes (exertional-Valsalva headaches, positional dizziness) | No | Spasmodic torticollis | Dystonic head tremor to the right (side-to-side) | Phasic dystonic head movements to the right with mild dystonic deviation to right. | Pachymeningeal enhancement, descent of cerebellar tonsil, flattening of anterior pons, | Conservative, avoiding provoking factors. | Gradual resolution of headaches at follow up visit in 5 years. | Gradual resolution of dystonia and tremor at follow up visit 5 years later. | None |
| 52/W (patient 4) | Yes (orthostatic headaches provoked by Valsalva, vertigo, tinnitus) | No | Gait unsteadiness, unspecified | Orthostatic mixed static and movement tremor of upper limbs, more on right. | No exam was mentioned in the article. | Pachymeningeal enhancement, descent of cerebellar tonsil, flattening of anterior pons, obliteration of perichiasmatic cistern, possible CSF leak at S1 level. | EBP every 6 weeks, later IV saline infusions every 3 weeks. S1,S2, and partial L5 hemilaminectomies did not identify definitive leak, area was packed with gelfoam and fibrin glue. | EBP led to transient marked improvement in headaches for 2 weeks. | Complete resolution for 2 weeks after EBP. After surgery, had relief for 6 weeks before return in symptoms. | Improvement but still persistent pachymeningeal enhancement | |
| Salazar et. al. 2016 | 68/M | Yes (occassional orthostatic headaches) | No | Gait unsteadiness | Bilateral progressive hand tremor over 2 years | Fast, distal kinetic tremor in B/L hands without postural/rest component. Mild dysmetria, truncal titubation, gait start hesitation, broad based gait with impaired tandem gait. | Diffuse infra- and supratentorial pachymeningitis, cerebellar tonsillar descent, mild brain sagging. No leak on CT myelogram. | Conservative (caffeine) | Not mentioned | Caffeine with partial response of symptoms. | Not done. |
| Iyer et al. | 23/M | Yes (orthostatic headaches) | No | Sleepiness in sitting position. | 7 month progressive right hand tremor, at rest and when holding objects. | Somnolent, pupillary light-near dissociation and restriction of upgaze. 3–5Hz Holmes tremor in R upper limb | Sagging of brain with transtentorial descent of third ventricle and diencephalon leading to deep brain swelling (more on left brainstem) and obliteration of basal cisterns. | Conservative (hydration, trendelenburg position) | Complete relief of somnolence and headaches. | Improved, with mild persistent kinetic and postural tremor at 1 week, complete resolution at 3 months. | Not done. |
[i] BSS: Brain sagging syndrome, CSF: Cerebrospinal fluid, SIH: Spontaneous intracranial hypotension, EBP: Epidural Blood patch.
Table 3
Case reports on SIH/BSS that have reported parkinsonism as predominant movement disorder.
| AUTHOR, YEAR | AGE/SEX | TYPICAL SIH SYMPTOMS NOTED? | TYPICAL FBSS SYMPTOMS NOTED? | OTHER SYMPTOMS | FEATURES OF PARKINSONISM | KEY EXAMINATION FINDINGS | IMAGING ABNORMALITIES | TREATMENT | IMPROVEMENT OF SIH/FBSS SYMPTOMS? | IMPROVEMENT OF MOVEMENT DISORDERS? | FOLLOW UP BRAIN IMAGING |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Pakiam et al. 1999 | 54/W | Yes (headaches worsened on cough, relieved on lying). | Depression that was treated. | Neck stiffness | Soft speech, R hand rest tremor, slowness in ADLs over 1 year, dysphagia | Hypophonia with weak gag, no rigidity, intermittent high frequency tremor, bradykinesia L > R, impaired tandem walk, retropulsion and impaired postural reflexes. | Downward displacement of posterior fossa structures. Elongation of brainstem in AP plane. Dural enhancement in posterior fossa. | EBP | Headaches resolved. | Resolution of symptoms with normal neurological exam by week 5. | Normal position of brainstem and cerebellar tonsils, mild persistence of midbrain elongation. |
| Mokri et al. 2014 | 78/F (patient 5) | Yes (no headache, but vertigo and nausea for 2 weeks) | Yes (slowness of thinking, memory difficulty) | – | Rest tremor of upper extremities | Rigidity of upper extremities, short step walking, hyperactive stretch reflexes, difficulty in abstraction and concentration. | Pachymeningeal enhancement, B/L subdural fluid collection. Low lying cerebellar tonsils. | None | At 4 months, cognitive functioned improved to “above average”. | No signs of parkinsonism at 4 moths. | Not done. |
| Gupta et al. 2021 | 66/W | No | Yes (personality changes, cognitive decline over 1 year). | – | Right hand tremor, gait slowness | Rest and re-emergent postural tremor of right hand. | Downward displacement of midbrain, cerebellar tonsils, diffuse dural enhancement. CSF Venous fistula at T9–T10 level. Normal PET scan. | Ligation of venous fistula | Reversal of cognition. | Substantial improvement after ligation. | Not available. |
| Cochran et al. 2021 | 64/W | No | Possible (drowsiness, depression) | Fatigue | Tremor in arms (R > L) and lips over 1 year, dysarthria | Bradykinesia (R > L), rest and postural tremor of right hand. Nasal speech, mild L facial weakness, dysmetria on finger-to-nose bilaterally. | Crowding of structures in suprasellar cistern, downward shift of optic chiasm, narrowing of 4th ventricle and decent of cerebellar tonsil. | Surgical repair of suspected dural leak at T7 which was repeated at 4 months. | Not mentioned. | After second repair, had sustained improvement of symptoms with no tremor, facial asymmetry, spasm or dysarthria. | Resolution of previous radiologic findings. |
| Frachet et al. 2023 | 21/W | Yes (headache, vomiting) | No | Left CN3 palsy, somnolence followed by coma | Right hemi parkinsonism (tremor and rigidity). | Acute stage: Left CN3 palsy, dilated pupils, comatose. After ICU stay: Right sided parkinsonism. | B/L subdural hematoma (L > R), collapse of 3rd ventricle, brain sagging, diffuse pachymeningeal enhancement. | Epidural blood patch | Not mentioned | Gradual resolution of symptoms | Not available. |
[i] AP: Anterior-posterior, BSS: Brain sagging syndrome, CSF: Cerebrospinal fluid, PET: Positron Emission Tomography, SIH: Spontaneous intracranial hypotension, ICU: Intensive Care Unit, EBP: Epidural Blood patch.
Table 4
Case reports on SIH/BSS that have reported chorea.
| AUTHOR, YEAR | AGE/SEX | TYPICAL SIH SYMPTOMS NOTED? | TYPICAL FBSS SYMPTOMS NOTED? | OTHER SYMPTOMS | DETAILS OF CHOREA | KEY EXAMINATION FINDINGS | IMAGING ABNORMALITIES | TREATMENT | IMPROVEMENT OF SIH/FBSS SYMPTOMS? | IMPROVEMENT OF MOVEMENT DISORDERS? | FOLLOW UP BRAIN IMAGING |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Mokri et al. 2006 | 59/M | Yes (orthostatic headaches, worsened on coughing, nausea) | Yes (memory complaints, confusion, sleepiness) | Dysarthria, dysphagia | Generalized chorea (face, trunk and extremities) | Worsening of chorea while walking, hyperkinetic dysarthria, and positive Babinski sign | Cerebellar tonsillar descent, T-2 hyperintensity of brainstem, pachymeningeal enhancement, CSF leak in cervico-thoracic area and T8–T9 meningeal diverticula | Epidural blood patch | Resolution of headaches, nausea, speech. Cognitive improvement not mentioned. | Complete resolution at 4 month follow up | None |
| Mulroy et al. 2017 | 42/M | Yes (6 months of chronic daily headache) | Yes (behavioral changes- impulsivity, disinhibtion. | Hiccups, Dysarthria, dysphagia | Limb and orofacial chorea and athetosis | Details are not mentioned | Both patients had downward displacement of brainstem and cerebellum. Slight distortion of basal ganglia in patient 1. Probable CSF Leak at T4 in patient 1. | Patient 1: conservatiive treatment, 2 EBP, T4 targeted blood patch | No improvement | No improvement | Not available. |
| 64/M | Yes (9 months of orthostatic headaches) | No | Limb and orofacial chorea and athetosis | Nasal speech | Patient 2: 2 epidural patches | No improvement | Temporary improvement in chorea but not sustained. | ||||
| Fearon et al. 2022 | 35/M | No | No | Decreased left hand dexterity, falls | Left sided hemichorea | Vertical supranuclear gaze impairment, brisk tendon reflexes, positive Babinski on left | Descent of brainstem, splenium, and cerebellar tonsils along with venous distention. MRI with thoracic epidural fluid collection. | EBP | – | Partial improvement in balance and chorea | Not available |
| Figueroa et al. 2018 | 62/M with Huntington’s Disease | Yes (progressive orthostatic headache with nausea and vomiting) | Yes- worsening of progresive cognitive decline and hallucinations | Gait disturbance | Pre-existing facial chorea got generalized | Details are not mentioned, but repeat MoCA with 5 point decrement to 23/30 | Pachymeningeal enhancement, sagging of brainstem, subdural hygromas, possible CSF leak at C1–C2 | Large volume blood patch (56 ml), and later subdural evacuation | Headache free, resumed work and MoCA back to baseline (29/30) at 3 months | Chorea returned to baseline | Small bilateral subdurals with resolution of brain slumping |
[i] BSS: Brain sagging syndrome, CSF: Cerebrospinal fluid, EBP: Epidural blood patch, SIH: Spontaneous intracranial hypotension.
Figure 1
Pathophysiology of movement disorders in brain sagging syndrome due to spontaneous intracranial hypotension: A. Vasogenic edema in subcortical structures such as the thalamus or putamen. B. Edema of the brainstem from venous stagnation. C. Reduction in angle between the vein of Galen and the straight sinus, creating a functional stenosis that worsens venous stagnation. D. Cerebellar tonsillar ectopy due to deep brain edema and cerebellar hypoperfusion (from venous hypertension) causing cerebellar symptoms.