When 'Idiopathic Intracranial Hypertension' Isn't Idiopathic
When 'Idiopathic Intracranial Hypertension' Isn't Idiopathic
A 23-year-old woman with idiopathic intracranial hypertension (IIH) presented with a recurrence of headache and visual disturbance. She had presented with similar symptoms 5 years earlier, when the diagnosis of IIH had been made, after magnetic resonance venography (MRV) had excluded cerebral venous sinus thrombosis (VST) (figure 1A,B). She had been asymptomatic for 18 months and had stopped her acetazolamide 6 months earlier.
(Enlarge Image)
Figure 1.
Magnetic resonance venography (MRV). (A) MR Venogram at time of diagnosis with IIH in 2007: flow signal is seen in the transverse and sigmoid sinuses bilaterally, although they are asymmetrical, the right side being dominant. (B) MR Venogram 2007. Lack of flow signal in the distal transverse sinuses (arrows) is the characteristic appearance seen in IIH. (C) MR Venogram at time of diagnosis with VST in 2012: there is no flow signal in the left transverse and sigmoid sinuses. (D) MR Venogram 2012. The characteristic IIH appearance remains on the right (arrow).
On examination, her visual acuity was 6/9 (right) and 6/15 (left). Her left visual field was constricted and there was bilateral papilloedema. Her cerebrospinal fluid (CSF) opening pressure was 80 cmH2O and we drained to a closing pressure of 22 cmH2O, resulting in complete resolution of both headache and visual disturbance. She was started on acetazolamide.
Within 48 h, her headaches returned with a new left-sided lower motor neurone seventh nerve palsy. We organised MR imaging with venography and then repeated her lumbar puncture. The opening pressure was now 29 cmH2O and she reported postprocedure improvement in her headache. However, within 24 h, her headache recurred, she developed a right lower motor neurone seventh nerve palsy (figure 2), bilateral sixth nerve palsies, neck stiffness, vomiting and visual disturbance. Magnetic resonance venography (MRV) showed a VST affecting the left transverse and sigmoid sinuses (figure 1C) and we started her on unfractionated heparin.
(Enlarge Image)
Figure 2.
Photographs of cranial nerve palsies.
In view of her markedly elevated opening pressure, background of IIH and the need for anticoagulation, we referred for early ventriculoperitoneal shunting. On the evening before her operation she became drowsy and confused. Repeat lumbar puncture gave an opening pressure of 98 cmH2O. All CSF pressures were measured with her lying in the lateral decubitus position.
The following morning she underwent uneventful ventriculoperitoneal shunt insertion and was subsequently reanticoagulated with unfractionated heparin, followed by low molecular weight heparin and eventually warfarin. Her right seventh nerve palsy and bilateral sixth nerve palsies improved during the first postoperative day, consistent with resolution of IIH. Her left seventh nerve palsy (figure 2) gradually improved in the months following discharge. We have not identified any thrombophilia tendency.
History
A 23-year-old woman with idiopathic intracranial hypertension (IIH) presented with a recurrence of headache and visual disturbance. She had presented with similar symptoms 5 years earlier, when the diagnosis of IIH had been made, after magnetic resonance venography (MRV) had excluded cerebral venous sinus thrombosis (VST) (figure 1A,B). She had been asymptomatic for 18 months and had stopped her acetazolamide 6 months earlier.
(Enlarge Image)
Figure 1.
Magnetic resonance venography (MRV). (A) MR Venogram at time of diagnosis with IIH in 2007: flow signal is seen in the transverse and sigmoid sinuses bilaterally, although they are asymmetrical, the right side being dominant. (B) MR Venogram 2007. Lack of flow signal in the distal transverse sinuses (arrows) is the characteristic appearance seen in IIH. (C) MR Venogram at time of diagnosis with VST in 2012: there is no flow signal in the left transverse and sigmoid sinuses. (D) MR Venogram 2012. The characteristic IIH appearance remains on the right (arrow).
On examination, her visual acuity was 6/9 (right) and 6/15 (left). Her left visual field was constricted and there was bilateral papilloedema. Her cerebrospinal fluid (CSF) opening pressure was 80 cmH2O and we drained to a closing pressure of 22 cmH2O, resulting in complete resolution of both headache and visual disturbance. She was started on acetazolamide.
Within 48 h, her headaches returned with a new left-sided lower motor neurone seventh nerve palsy. We organised MR imaging with venography and then repeated her lumbar puncture. The opening pressure was now 29 cmH2O and she reported postprocedure improvement in her headache. However, within 24 h, her headache recurred, she developed a right lower motor neurone seventh nerve palsy (figure 2), bilateral sixth nerve palsies, neck stiffness, vomiting and visual disturbance. Magnetic resonance venography (MRV) showed a VST affecting the left transverse and sigmoid sinuses (figure 1C) and we started her on unfractionated heparin.
(Enlarge Image)
Figure 2.
Photographs of cranial nerve palsies.
In view of her markedly elevated opening pressure, background of IIH and the need for anticoagulation, we referred for early ventriculoperitoneal shunting. On the evening before her operation she became drowsy and confused. Repeat lumbar puncture gave an opening pressure of 98 cmH2O. All CSF pressures were measured with her lying in the lateral decubitus position.
The following morning she underwent uneventful ventriculoperitoneal shunt insertion and was subsequently reanticoagulated with unfractionated heparin, followed by low molecular weight heparin and eventually warfarin. Her right seventh nerve palsy and bilateral sixth nerve palsies improved during the first postoperative day, consistent with resolution of IIH. Her left seventh nerve palsy (figure 2) gradually improved in the months following discharge. We have not identified any thrombophilia tendency.
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