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Intracranial Hypotension With Parkinsonism, Ataxia, and Bulbar Weakness FREE

Anthony S.-I. Pakiam, MD; Christine Lee, MD; Anthony E. Lang, MD
[+] Author Affiliations

From the Division of Neurology (Drs Pakiam and Lang) and the Department of Medicine (Dr Lee), Toronto Western Hospital, Toronto, Ontario.


Arch Neurol. 1999;56(7):869-872. doi:10.1001/archneur.56.7.869.
Text Size: A A A
Published online

ABSTRACT

Objective  To describe a case of spontaneous intracranial hypotension with a previously unreported constellation of presenting features.

Design  Case report.

Setting  Tertiary care center.

Main Outcome and Results  We describe a patient with intracranial hypotension who presented with a parkinsonian syndrome and later development of ataxia and prominent bulbar symptomatology. Headache was not a feature of her initial presentation and was only reported after repeated questioning during later evaluations. Magnetic resonance imaging of the patient's head revealed findings characteristic of intracranial hypotension. An [18F]fluoro-m-tyrosine positron emission tomographic scan showed normal striatal activity, suggesting intact presynaptic nigrostriatal function. Opening pressure on lumbar puncture was reduced at 40 mm H2O. A source of cerebrospinal fluid leakage was not identified on nuclear cisternography and the patient underwent lumbar epidural blood patching, which resulted in complete resolution of her signs and symptoms as well as in a marked improvement in her imaging findings.

Conclusions  The clinical spectrum of intracranial hypotension can be broadened to include parkinsonism, cerebellar ataxia, and prominent bulbar dysfunction. As with more common manifestations of the disorder, these features may resolve after appropriate treatment.

Figures in this Article

INTRACRANIAL hypotension15 characteristically presents with a postural headache and low cerebrospinal fluid (CSF) pressure. Common accompanying features include nausea, vomiting, stiff neck, photophobia, and vertiginous symptoms. The syndrome may occur spontaneously, as first described by Schaltenbrand,6 follow rupture of an arachnoid (Tarlov) cyst, or have a precipitant, such as lumbar puncture, trauma, or cranial or spinal surgery.13 We describe a patient with an unusual constellation of presenting features in whom headache was not initially a prominent complaint.

REPORT OF A CASE

A 54-year-old right-handed woman was seen in our clinic regarding the possibility of Parkinson disease. One year earlier, she had experienced the onset of a right hand tremor that had mainly been present at rest but had spontaneously improved before her clinic visit. Also, her speech had become softer during the past year, and certain activities, such as eating or walking, were slower than before. She had noted no change in speed or ability to perform other activities, including the use of utensils, personal hygiene, and getting dressed or undressed. Writing had become extremely "sloppy" but not micrographic. She had experienced 3 falls, 1 as a result of tripping over an object and 2 from losing her balance while walking downhill. She had been treated for depressive symptoms with paroxetine (10 mg/d) for 8 months, with some improvement in her mood. The paroxetine therapy had been discontinued 3 months earlier. She was hospitalized 2 months after her initial clinic visit and had in the interim developed more of a nasal quality to her voice; dysphagia, which was more prominent with liquids than solids; and vomiting 2 or 3 times per week. She also admitted to a mild headache, which was holocephalic, steady, and pressurelike in quality and occurred only when she coughed. Standing up did not precipitate her headaches, but lying down could make them less intense. She also reported episodic fatigue and having been "uptight" and making mistakes at work 2 to 3 years earlier. Also, in retrospect, she checked some notes that she had taken 312 years earlier and found that she had experienced a sensation of a "plugged" left ear 1 day after doing a handstand in a swimming pool and had headaches 4 days later. The following year, in addition to having difficulty at work, she had again noted a sensation of plugged ears. She had no visual, vertiginous, or autonomic symptoms; she had no history of head or neck trauma; and she had never had a lumbar puncture. Her only medication was alprazolam (0.25 mg nightly). There was a history of a tremor in her father, a paternal aunt, and 2 cousins. Her medical history was remarkable for breast cancer 5 years earlier, for which she had undergone a left mastectomy and had subsequently been treated with tamoxifen citrate, with no evidence of recurrence.

General examination revealed a tall, thin woman. Neurological examination revealed normal alertness and cognition. Her speech had a nasal quality and was hypophonic. Her palatal elevation was reduced and her gag reflex was weak. The findings of the remainder of her cranial nerve examination were normal. Myerson sign was absent. Muscle strength and tone were normal, with no cogwheeling. Only a very mild, intermittent high-frequency tremor was present in the extremities in all positions. Fast, repetitive movements demonstrated mild but definite bradykinesia, more so on the left than on the right. No dysmetria was present, and sensation was intact to all modalities. Her deep tendon reflexes were normal except for reduced ankle reflexes, and her plantar responses were flexor. Her gait was unsteady, and she was unable to tandem walk, although she could ambulate without support. Her postural reflexes were impaired, with several steps of retropulsion but no falling.

Magnetic resonance imaging (MRI) of the brain (Figure 1) revealed a downward displacement of posterior fossa structures and an elongation of the brainstem in the anteroposterior plane. Dural enhancement was present mainly in the posterior fossa. The findings of [18F]fluoro-m-tyrosine positron emission tomographic scanning were normal. Opening pressure on lumbar puncture was low at 40 mm H2O, and the results of CSF studies, including cytology, were otherwise normal. Cisternography using technetium pentetic acid failed to show a CSF leak after lumbar instillation. The patient underwent epidural blood patching with 20 mL of blood in the midlumbar area, after which she remained supine for 30 minutes but was not placed in the head-down position. After the blood patching, her voice and swallowing returned to normal within 2 days. Her headaches resolved rapidly. The neck stiffness, which had developed after the lumbar puncture, also resolved after the blood patching. Five weeks later, the findings of her neurological examination were normal. Her palatal elevation and gag reflex were normal, as were her walking ability and performance of rapid repetitive movements. Her writing skills also returned to normal. Follow-up MRI (Figure 2) after 7 weeks showed a normal position of the brainstem and cerebellar tonsils. There was mild persistence of the elongation of the midbrain in the anteroposterior direction.

Place holder to copy figure label and caption
Figure 1.

Pretreatment magnetic resonance imaging scan of the patient's brain. A, Sagittal T1-weighted scan showing low-lying cerebellar tonsils. B, Axial T2-weighted scan showing effacement of infratentorial cisterns and elongation of the brainstem in the anteroposterior plane. C, Axial gadolinium–pentetic acid–enhanced T1-weighted scan showing linear dural enhancement in the posterior fossa.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Magnetic resonance imaging scan of the patient's brain 5 weeks after treatment. A, Sagittal T1-weighted scan showing a return of the cerebellar tonsils to a normal position. B, Axial T2-weighted scan showing an increase in cisternal space surrounding the midbrain and mild persistent deformity.

Graphic Jump Location

COMMENT

Intracranial hypotension has been reported to occur not only after significant trauma, spinal or intracranial surgery, and lumbar puncture, but also after even minor trauma or strain. The syndrome may follow or occur at the time of sexual intercourse,7 paroxysmal coughing,3 bending down,8 or falling and landing on the buttocks,1 in association with back pain radiating into the lower extremities. In the latter case, CSF leakage is presumably precipitated by trauma causing a disruption in the nerve root sleeve at the lumbosacral level. Similarly, cases have been associated with repetitive strenuous upper extremity motions, such as racket sports9 or throwing movements when fishing,3 suggesting injury to nerve root sheaths at the level of nerve roots supplying the brachial plexus, through which stretch is transmitted. Our patient experienced minor strain the day before her first symptoms occurred, but there was no clear precipitating event, so the diagnosis in her case is best classified as spontaneous intracranial hypotension.

Our patient had an unusual presentation of spontaneous intracranial hypotension and was initially referred with a query of Parkinson disease because of a unilateral resting hand tremor, bradykinesia, and gait imbalance. Another atypical feature was the subsequent development of bulbar weakness manifesting as nasality of speech and dysphagia. Cranial nerve abnormalities reported in other cases of intracranial hypotension have included symptoms referable to the optic nerve or chiasm manifesting as blurred vision,3,10 transient visual obscurations, and visual field deficits.8,11 Abducens palsy11 with resultant horizontal diplopia may be present, most likely as the result of distortion of the nerve from traction or pressure. Symptoms and findings referable to cranial nerve VIII,3,5,8,12 including sensorineural hearing loss, hyperacusis, tinnitus, vertigo, and a sensation of "fullness",3 "popping,"5 or "echoing"4 in the ear, have also been reported. Some of these latter symptoms may be related to secondary alterations in intralabyrinthine pressure dynamics, which could also account for the sensation of "ear plugging" experienced by our patient. Finally, facial weakness3,13 or numbness3 and dysgeusia3 have been reported in association with intracranial hypotension. Cranial nerve palsies may be persistent for a prolonged period, despite immediate relief of postural headache after epidural blood patching.13

Potential pathophysiological mechanisms contributing to cranial nerve dysfunction in intracranial hypotension include intracranial venous distention, given the fixed volume of the intracranial space, and traction or pressure on affected cranial nerves, especially when downward displacement of the brainstem is evident, as was the case in our patient. The cardinal symptom of postural headache is also postulated to result from traction on pain-sensitive structures, including cranial nerves,2 rather than from the reduced intracranial pressure alone, since maneuvers to increase intracranial pressure, such as jugular venous compression or valsalva, increase rather than relieve the headache.4 The degree of brain descent and resultant contributory traction mechanism to the characteristic postural dependence of headache and other symptoms may be somewhat underestimated by MRI, since these images are by necessity obtained with the patient in the recumbent position. In addition to a traction mechanism affecting cranial nerves and pain-sensitive structures in the posterior fossa, a compressive effect on the brainstem can be postulated in light of the midbrain deformity present in our patient, in whom one manifestation was a partial parkinsonian syndrome. However, an [18F]fluoro-m-tyrosine positron emission tomographic scan14 did not provide evidence of the speculated presynaptic dopaminergic dysfunction of nigral origin. Depressed level of consciousness, described in a recent report of intracranial hypotension due to a torn dural root sleeve and ruptured Tarlov cyst, was also blamed on central herniation and compression of diencephalic structures.15

Abnormal findings on brain MRI in our patient were characteristic of intracranial hypotension and consisted of low-lying cerebellar tonsils; displacement of the brainstem, with effacement of the interpeduncular and prepontine cisterns; and thickening of the meninges with linear dural enhancement, which were predominant in the posterior fossa. Subdural hematomas4 or subdural effusions and dilatation of cortical and spinal epidural veins resulting from the altered pressure dynamics may also be seen,5,12 and dural enhancement is characteristically diffuse.1619 Cerebrospinal fluid pressure is typically,2 but not always,20 reduced to less than 60 mm H2O, and CSF analysis may disclose a variable presence of erythrocytosis, lymphocytic pleocytosis, and an elevated protein content.3,21 The site of CSF leakage was sought in our patient by nuclear cisternography, the findings of which were unrevealing. Alternative studies include spinal MRI and computed tomographic myelography, which is potentially more sensitive3 but was not performed in our patient, since the diagnostic lumbar puncture was not well tolerated. Recently, Pleasure et al15 emphasized the utility of thin-section MRI of the entire spine in refractory cases. Treatment may involve surgical ligation or epidural packing of leaking meningeal diverticula if found.3,22 However, many investigators would first try a large-volume (20-30 mL) epidural blood patch in the lumbar region, followed by a 5-minute period with the patient's head lowered 30°.15 In our patient, blood patching provided complete and rapid relief of symptoms along with radiographic improvement. When the initial blood patching fails to result in sustained benefit, subsequent investigations and/or another blood patching at the same or a higher level can be performed.5,10 Alternatively, a 24-hour epidural infusion of normal saline may be effective.4

In conclusion, the clinical spectrum of intracranial hypotension can now be broadened to rarely include parkinsonism, cerebellar ataxia, and prominent bulbar symptomatology. As with other symptoms more commonly present in this condition, these features and accompanying radiological changes may resolve following appropriate treatment of the disorder.

ARTICLE INFORMATION

Accepted for publication August 17, 1998.

The [18F]fluoro-m-tyrosine positron emission tomographic scanning was performed by Claude Nahmias, PhD.

Reprints: Anthony E. Lang, MD, Division of Neurology, Toronto Western Hospital, 399 Bathurst St, MP 11-306, Toronto, Ontario, Canada M5T 2S8.

REFERENCES

Bell  WEJoynt  RJSahs  AL Low spinal fluid pressure syndromes. Neurology. 1960;10512- 521
Link to Article
Khurana  RK Intracranial hypotension. Semin Neurol. 1996;165- 10
Link to Article
Mokri  BPiepgras  DGMiller  GM Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc. 1997;72400- 413
Link to Article
Rando  TAFishman  RA Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology. 1992;42481- 487
Link to Article
Renowden  SAGregory  RHyman  NHilton-Jones  D Spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 1995;59511- 515
Link to Article
Schaltenbrand  G Neuere Anschauungen zur pathophysiologie der Liquorzirkulation. Zentralb Neurochir. 1938;3290- 300
Paulson  GWKlawans  HL Benign orgasmic cephalgia. Headache. 1974;13181- 187
Link to Article
Weitz  SRDrasner  K Spontaneous intracranial hypotension. Anesthesiology. 1996;85923- 925
Link to Article
Garcia-Albea  ECabrera  FTejeiro  JJiminez-Jiminez  F-JVaquero  A Delayed postexertional headache, intracranial hypotension and racket sports. J Neurol Neurosurg Psychiatry. 1992;55975
Link to Article
Benzon  HTNemickas  RMolloy  REAhmad  SMelen  OCohen  B Lumbar and thoracic epidural blood injections to treat spontaneous intracranial hypotension. Anesthesiology. 1996;85920- 922
Link to Article
Horton  JCFishman  RA Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology. 1994;101244- 251
Link to Article
Pannullo  SCReich  JBKrol  GDeck  FPosner  JB MRI changes in intracranial hypotension. Neurology. 1993;43919- 926
Link to Article
Dunbar  SAKatz  NP Failure of delayed epidural blood patching to correct persistent cranial nerve palsies. Anesth Analg. 1994;79806- 807
Link to Article
Nahmias  CWahl  LRaman  CFirnau  GGarnett  ES A probe for intracerebral aromatic amino-acid decarboxylase activity: distribution and kinetics of [18F]6-fluoro-L-m-tyrosine in the human brain. Mov Disord. 1995;10298- 304
Link to Article
Pleasure  SJAbosch  AFriedman  J  et al.  Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology. 1998;501854- 1857
Link to Article
Fishman  RADillon  WP Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology. 1993;43609- 611
Link to Article
Hochman  MSNaidick  TPKobetz  SAFernandez-Maitin  A Spontaneous intracranial hypotension with pachymeningeal enhancement on MRI. Neurology. 1992;421628- 1630
Link to Article
River  YSchwartz  AGomoria  JMSoffer  DSegal  T Clinical significance of diffuse dural meningeal enhancement detected by magnetic resonance imaging. J Neurosurg. 1996;85777- 783
Link to Article
Sable  SGRamadan  NM Meningeal enhancement and low CSF pressure headache: an MRI study. Cephalalgia. 1991;11275- 276
Link to Article
Not Available, Case records of the Massachusetts General Hospital: weekly clinicopathological exercises. N Engl J Med. 1998;338180- 188Case 2-1998
Link to Article
Mokri  BParisi  JEScheithauer  BWPiepgras  DGMiller  GM Meningeal biopsy in intracranial hypotension: meningeal enhancement on MRI. Neurology. 1995;451801- 1807
Link to Article
Schievink  WIMeyer  FBAtkinson  JLDMokri  B Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg. 1996;84598- 605
Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.

Pretreatment magnetic resonance imaging scan of the patient's brain. A, Sagittal T1-weighted scan showing low-lying cerebellar tonsils. B, Axial T2-weighted scan showing effacement of infratentorial cisterns and elongation of the brainstem in the anteroposterior plane. C, Axial gadolinium–pentetic acid–enhanced T1-weighted scan showing linear dural enhancement in the posterior fossa.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Magnetic resonance imaging scan of the patient's brain 5 weeks after treatment. A, Sagittal T1-weighted scan showing a return of the cerebellar tonsils to a normal position. B, Axial T2-weighted scan showing an increase in cisternal space surrounding the midbrain and mild persistent deformity.

Graphic Jump Location

Tables

References

Bell  WEJoynt  RJSahs  AL Low spinal fluid pressure syndromes. Neurology. 1960;10512- 521
Link to Article
Khurana  RK Intracranial hypotension. Semin Neurol. 1996;165- 10
Link to Article
Mokri  BPiepgras  DGMiller  GM Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc. 1997;72400- 413
Link to Article
Rando  TAFishman  RA Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology. 1992;42481- 487
Link to Article
Renowden  SAGregory  RHyman  NHilton-Jones  D Spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 1995;59511- 515
Link to Article
Schaltenbrand  G Neuere Anschauungen zur pathophysiologie der Liquorzirkulation. Zentralb Neurochir. 1938;3290- 300
Paulson  GWKlawans  HL Benign orgasmic cephalgia. Headache. 1974;13181- 187
Link to Article
Weitz  SRDrasner  K Spontaneous intracranial hypotension. Anesthesiology. 1996;85923- 925
Link to Article
Garcia-Albea  ECabrera  FTejeiro  JJiminez-Jiminez  F-JVaquero  A Delayed postexertional headache, intracranial hypotension and racket sports. J Neurol Neurosurg Psychiatry. 1992;55975
Link to Article
Benzon  HTNemickas  RMolloy  REAhmad  SMelen  OCohen  B Lumbar and thoracic epidural blood injections to treat spontaneous intracranial hypotension. Anesthesiology. 1996;85920- 922
Link to Article
Horton  JCFishman  RA Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology. 1994;101244- 251
Link to Article
Pannullo  SCReich  JBKrol  GDeck  FPosner  JB MRI changes in intracranial hypotension. Neurology. 1993;43919- 926
Link to Article
Dunbar  SAKatz  NP Failure of delayed epidural blood patching to correct persistent cranial nerve palsies. Anesth Analg. 1994;79806- 807
Link to Article
Nahmias  CWahl  LRaman  CFirnau  GGarnett  ES A probe for intracerebral aromatic amino-acid decarboxylase activity: distribution and kinetics of [18F]6-fluoro-L-m-tyrosine in the human brain. Mov Disord. 1995;10298- 304
Link to Article
Pleasure  SJAbosch  AFriedman  J  et al.  Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology. 1998;501854- 1857
Link to Article
Fishman  RADillon  WP Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology. 1993;43609- 611
Link to Article
Hochman  MSNaidick  TPKobetz  SAFernandez-Maitin  A Spontaneous intracranial hypotension with pachymeningeal enhancement on MRI. Neurology. 1992;421628- 1630
Link to Article
River  YSchwartz  AGomoria  JMSoffer  DSegal  T Clinical significance of diffuse dural meningeal enhancement detected by magnetic resonance imaging. J Neurosurg. 1996;85777- 783
Link to Article
Sable  SGRamadan  NM Meningeal enhancement and low CSF pressure headache: an MRI study. Cephalalgia. 1991;11275- 276
Link to Article
Not Available, Case records of the Massachusetts General Hospital: weekly clinicopathological exercises. N Engl J Med. 1998;338180- 188Case 2-1998
Link to Article
Mokri  BParisi  JEScheithauer  BWPiepgras  DGMiller  GM Meningeal biopsy in intracranial hypotension: meningeal enhancement on MRI. Neurology. 1995;451801- 1807
Link to Article
Schievink  WIMeyer  FBAtkinson  JLDMokri  B Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg. 1996;84598- 605
Link to Article

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