A case of unusual blindness

Surya Sreeram Palakuru MD, Lamya Boujelbane MD, MPH,

Sowmya Korapati MD, Aurora Health Care Internal Medicine

Residency Program.

The Case ●  35 year old male who presented with ○  Bilateral complete vision loss - 6 day duration. ○  It started as blurry vision while vacationing in

Antigua and progressed to complete vision loss in 24 hrs.

History (Contd.) ●  Review of Systems ○  Positive for pressure like sensation behind the eyes ○ Negative for diplopia, discharge, pain.

●  Relevant History ○ Denies any history of ■ Recent ear infections ■ Treatment for acne ■ Vitamin A supplement use ■  Lead exposure ■ Seizure disorder ■ Steroid use

History (Contd.) ●  Past Medical History ○ ADHD

●  Allergies ○ None

●  Medications ○ Adderall

●  Family History ○ Non contributory

●  Social History ○ Non smoker. Drinks alcohol occasionally. No

recreational drug use. Works as a software engineer.

Physical Examination ●  Vitals: HR 56, BP 137/83, RR 20, POx 97% on RA, T

98.6 ●  General: Anxious, overweight young male ●  Respiratory: Clear to auscultation ●  Cardiovascular: RRR. S1 and S2 present. ●  Ophthalmic: ○ No light perception ○ IOP and Anterior segment - WNL ○ Pupils round, equal but non reactive to light ○ Fundoscopy: Bilateral papilledema with retinal

hemorrhage and venous tortuosity ●  Neurological: Motor, Sensory and Cerebellar - WNL.

Labs

●  AST - 18 U/L, ALT - 24 U/L, Alk Phos - 101 U/L ●  ESR - 16 mm/hr ●  CRP - 1.8 mg/dl ●  ACE level - 24 U/L

88 7.2 14.1

246 43

24 108 142

4.4 26 1.0 4.4 26 1.0

Imaging

●  MRI, MRA and MRV were within normal limits.

Labs (Contd.)

CSF Analysis ●  Opening Pressure - 330 mm of H2O ●  Appearance - clear and colorless ●  Protein - 34 mg/dl ●  Glucose - 64 mg/dl ●  Gram stain - Negative ●  Cytology - 2 mononuclear WBC

Negative CSF Workup ●  Bacterial culture ●  Viral culture ●  Fungal culture ●  VDRL ●  HSV1 ●  HSV 2 ●  West Nile Virus IgM ●  Varicella Zoster PCR ●  Toxoplasma PCR ●  Cryptococcal antigen ●  B.burgdorferi Ig

●  Based on the clinical picture and investigations secondary causes of raised ICP like intracranial mass lesions (tumor, abscess), choroid plexus papilloma, arachnoid granulation adhesions after subarachnoid hemorrhage or bacterial meningitis, obstructive hydrocephalus, venous sinus thrombosis were ruled out.

●  Other differentials like glaucoma, optic neuritis, multiple sclerosis were ruled out.

Pseudotumor Cerebri

Discussion ●  Pseudotumor cerebri (PTC)/Idiopathic intracranial

hypertension (IIH)/Benign Intracranial hypertension, is a disorder of unknown etiology, first described by Quincke in 1897. (1)

●  It is characterized by symptoms and signs of increased intracranial pressure, elevated intracranial pressure with normal CSF composition and no identifiable etiology of neuroradiologic studies. (2)

●  The incidence of IIH ranges from 0.9-1 per 100,000 in the general population (3). More than 90% of PTC cases are seen in obese women of child bearing age (4).

●  Pathogenesis is unclear.

Clinical Presentation Symptoms

■  Headache (non specific and variable) (8),

■  Horizontal diplopia (CN VI palsy),

■  Pulsatile tinnitus

■  Vision loss (acute or progressive), transient visual obscuations.

Physical exam

o  Papilledema

o  Visual field defects (usually peripheral)

o  Abducens palsy (unilateral or bilateral).

Diagnosis ●  MRI with/without contrast

with post contrast MR venography is the imaging modality of choice. ○  Flattening of the

posterior sclera is the most reliable indicator on MRI (9-10) ■  Specificity - 100% ■  Sensitivity - 43.5%

○  Lumbar puncture ■  Opening pressure of

>250 mm of H2O. ○  CSF analysis - Normal

Associations... ●  Medications and

exogenous substances ○ Growth hormone ○ Tetracycline ○ Retinoids ○ Nalidixic acid ○  Lithium, ○ Nitrofurantoin ○ Thyroid replacement ○ Corticosteroid

withdrawal.

●  Associated systemic illnesses ○ Addison’s disease ○ Hypoparathyroidism ○ Anemia ○  Polycystic Ovarian

Syndrome ○ Systemic Lupus

Erythematosus ○ Behcet’s syndrome

●  Our patient did not have any associated systemic illnesses.

●  Only medication he was taking was Adderall for ADHD.

Adderall ●  Adderall (Dextroamphetamine and amphetmine)

is a non catecholamine, sympathomimetic amine that promotes release of catecholamines from presynaptic nerve terminals.

●  Adderall XR was approved to treat ADHD in adults in 2004.

Reports

●  Based on reports from FDA, since 2003 there were 6 cases of people using Adderall who developed pseudotumor cerebri

●  Females - 4 ●  Males - 2 ●  Age range

○  10-19 yrs - 4 cases ○  30-39 yrs - 2 cases

Treatment ●  Treatment goals ○ Alleviation of symptoms ○ Preservation of vision

●  Initial Treatment ○ Stop potential agents that can cause IIH. ○ Acetazolamide ○ Lasix and topiramate ○ Short term steroids/serial lumbar punctures ■ Bridge to definitive surgical therapy in

patients with rapidly progressive symptoms.

Treatment (Contd.) ●  Surgical Treatment ○ Optic nerve sheath fenestration (ONSF) ■ Less severe side effects and significantly

better outcomes(11). ■ Benefits may not be long lasting (12).

○ CSF shunts (Lumboperitoneal/Ventricular) ■ Failure of maximal medical therapy/ONSF ■ Intractable headaches ■ Available expertise ■ Patient preference.

●  Newer modalities ○ Intracranial dural venous stenting.

Prognosis ●  The prognosis for visual outcome in IIH is generally good.

Prompt intervention results in a favorable outcome(13). ●  People at higher risk of permanent visual loss ○ Males ○ Younger age ○ Black race ○ Systemic hypertension ○ Anemia ○ Significant vision loss at presentation ○ Obesity ○ Higher opening pressures

●  Recurrence of symptoms occur in 8-38% of patients

Back to the Patient... ●  Adderall was stopped. ●  With lumbar puncture, pressure sensation behind his eyes

was relieved and his vision improved slightly. ●  He was started on acetazolamide 250 q6h and also on

steroids initially(for suspicion of multiple sclerosis). ●  Steroids were later tapered off and discontinued after

negative workup for MS. ●  An LP shunt was placed due to the severity of visual loss

with continuous, gradual improvement in his vision. ●  Post discharge his vision improved dramatically with

almost complete resolution of his symptoms at 1 month follow up.

References 1.  Quincke H. Üeber Meningitis serosa und verwandte Zustände. Dtsch Z Nervenheilk. 1897;149–168. 2.  Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59:1492. 3.  Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. Jun 2004;24(2):138-45. 4.  Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. Mar 2008;8(2):

87-93.

5.  Bruce BB, Kedar S, Van Stavern GP, Monaghan D, Acierno MD, Braswell RA, et al. Idiopathic intracranial hypertension in men. Neurology. Jan 27 2009;72(4):304-9.

6.  Bateman GA, Stevens SA, Stimpson J. A mathematical model of idiopathic intracranial hypertension incorporating increased arterial inflow and variable venous outflow collapsibility. J Neurosurg. Mar 2009;110(3):446-56.

7.  Bateman GA. Arterial inflow and venous outflow in idiopathic intracranial hypertension associated with venous outflow stenoses. J Clin Neurosci. Apr 2008;15(4):402-8.

8.  González-Hernández A, Fabre-Pi O, Díaz-Nicolás S, López-Fernández JC, López-Veloso C, Jiménez-Mateos A. [Headache in idiopathic intracranial hypertension]. Rev Neurol. Jul 1-15 2009;49(1):17-20.

9.  Agid R, Farb RI, Willinsky RA, Mikulis DJ, Tomlinson G. Idiopathic intracranial hypertension: the validity of cross-sectional neuroimaging signs. Neuroradiology. Aug 2006;48(8):521-7.

10.  Maralani PJ, Hassanlou M, Torres C, Chakraborty S, Kingstone M, Patel V, et al. Accuracy of brain imaging in the diagnosis of idiopathic intracranial hypertension. Clin Radiol. Jul 2012;67(7):656-63.

11.  Feldon SE. Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension. Neurosurg Focus. 2007;23(5):E6.

12.  Chandrasekaran S, McCluskey P, Minassian D, Assaad N. Visual outcomes for optic nerve sheath fenestration in pseudotumour cerebri and related conditions. Clin Experiment Ophthalmol. Sep-Oct 2006;34(7):661-5.

13.  Rowe FJ, Sarkies NJ. Visual outcome in a prospective study of idiopathic intracranial hypertension. Arch Ophthalmol 1999; 117:1571.