Neurology Case Presentation

Scott M. Shorten, MDPGY-3

Right-handed man

CC: right facial droop, right arm and leg tingling and weakness

HPI• recurrent drooping of the right face• started 1.5 yrs ago without clear precipitant• multiple times per day and while asleep, no warning, no

trigger• Average 30 minutes (5 min-2 hours), with complete recovery

between• Sometimes associated hand/arm numbness, no other

consistent symptoms• This episode concerning due to ‘stabbing’ mid-frontal

headache with photo/phonophobia, left arm and leg weakness, and lasted over 2 hours. Onset while out in the heat gardening.

• ROS: fatigue, chest discomfort, neck pain

PMHx/SurgHx

• COPD• Hyperlipidemia• Depression• Septic thrombophlebitis, R Cephalic vein

• Appendectomy• Hemorrhoidectomy

Family History

• Mother: Bell’s Palsy, Thyroid disease• Father: Meniere’s Disease• Grandmother: Stroke

Soc Hx

• Married, lives locally • Diesel mechanic• Smokes 1ppd x 30 years• No use of EtOH or Recreational Drugs

Medications

• Verapamil 60mg TID • Carbamazepine 200mg BID• Aspirin 325 qD• Famotidine 10mg qD• Trandolapril 2mg qD• Multivitamin• Simvastatin 40mg qHS• Albuterol PRN

• Allergy: Minocycline

VS: 132/80 36.6 p67 r18

GEN: alert, cooperative, pleasant, NAD.

CV, Pulm, MSK examinations normal

MS: oriented to person/place/time/situation

Speech: slight labial dysarthria. Language normal.

CN: NLF flattened on the right, decreased pinprick Right V1-3*

Motor: Tone and bulk normal, 5/5 throughout

Sensory: decreased pinprick Right UE & LE

Reflexes:

Coordination: normal F-N-F and Heel-shinGait: normal x4, no Romberg

2 22 22 2

1

3

1~ ~

3

?

Workup(occurred over ~1 year)

• Imaging: – MRI of complete neuro-axis: normal– CTA head and neck: normal– Trans-esophageal Echocardiogram: normal– 4-vessel angiogram normal

• Prolonged and Video EEG negative for epileptic event, no slowing, no change on trial of Keppra

• PET: Left lower lobe infiltrate likely pneumonia, no neoplasm

Lumbar Punctures:RBCs WBCs Prot Glu

Presentation 90 20 (88%L) 62 492 days later 2750 15 (51%L) 80 597 days later 140 10 (77%L) 83 601 month later 1 2 70 607 months later1 2 51 6313 months later550 33 (94%L) 76 60

• No growth of bacteria or fungus• Cryptococcal Ab: negative• Oligoclonal bands: negative• IgG index 0.59• ACE: <4• Cytology: negative x4• Extensive workup with ID: unremarkable• Autoimmune/paraneoplastic workup: normal• DRVVT + on 3 months after presentation but normal on

subsequent 6 months later: “possible transient due to viral infection”

• EBV studies: +Capsid IgG +Nuclear ag ab +Early ag ab; - Capsid IgM

??

Mollaret’s Meningitisv.

Pseudomigraine with Temporary Neurologic Symptoms and Lymphocytic Pleocytosis

• started empiric treatment with Acyclovir IV, then Valacyclovir 1000mg daily x 1 year

• Increased verapamil for continued possibility of vasospasm

Mollaret, P. Revue Neurologique. 1944 .Shalabi, M. Clinical Infectious Diseases. 2006.

Mollaret’s Meningitis• Described in 1944• >3 episodes of fever and

meningismus; weeks to years between

• Lasting 2-5 days, wide variation

• Spontaneous resolution• ~50% with neurologic

features

Pierre Mollaret (1898-1987)

• Most commonly due to HSV-2, often with muco-cutaneous lesions found elsewhere

• Diagnosis confirmed with CSF HSV PCR• Valacyclovir prevented genital lesion recurrence

in first year, but no change in meningitis frequencyCanadian Medical Assn. http://www.cmaj.ca/content/174/12/1710.2/F2.expansion.html

Ginsberg L. Pract Neurol 2008;8:348-361 Aurelius E. Clinical Infectious Diseases .2012.

Pseudomigraine with Temporary Neurologic Symptoms and Lymphocytic Pleocytosis

=Migrainous Syndrome with CSF Pleocytosis

=Syndrome of Transient Headache and Neurologic Deficits with CSF Pleocytosis (HaNDL)

HaNDL• First described in 1981• Self-limited, benign condition• Transient neurological deficits

- 15 minutes to 2 hours each, over weeks-months

• Moderate-Severe throbbing headache• Lymphocyte predominant pleocytosis– Avg 199 cells (range 10-760), most >90% Lymph; – avg protein 96, elevated in 96%– Glucose normal– Opening pressure elevated in ~50%

Bartleson, JD. Neurology. 1981Gomez-Aranda, F. Brain. 1997

• Usually in 30s-40s (range 7-52 yrs)• 25-40% had preceding

cough/rhinitis/fatigue/diarrhea• No consistent gender predominance

• Neuroimaging is usually normal– Leptomeningeal enhancement– Hypoperfusion on CT perfusion

• EEG generally shows slowing in the corresponding region

Other Studies

HaNDL Etiology

• Inflammatory/Infectious?– Few reports; Echovirus, HHV-6.

• Migrainous?– SPECT imaging with decreased blood flow at sites

corresponding to neurologic deficit– spreading cortical depression phenomenon

• Infectious, triggering cortical depression?

Castels-van Daele, M. Lancet. 1981.Emond, H. Cephalalgia. 2009.Caminero, AB. Headache. 1997

Diagnosis / Tx• Must first exclude more sinister causes• CSF with >15 cells/mL of lymphocyte

predominance• Episodes of moderate-severe headache

occurring with or shortly following symptoms• Episodes recurring within 3 months

• Symptomatic treatment only, if needed

The International Classification of Headache Disorders: Cephalalgia. 2004

Our Patient

• frequency of attacks 3-4 per day (from up to 20).

• Mostly affecting only his right face• Usually associated with moderate headache• Happy with improvement

Shalabi M, Whitley RJ. Recurrent benign lymphocytic meningitis. Clinical Infect Dis. 2006;43(9):1194.

L Ginsberg, J Kidd. Chronic and Recurrent Meningitis. Pract Neurol 2008;8:348-361.

Aurelius E, Franzen-Röhl E, Glimåker M. Long-term valacyclovir suppressive treatment after herpes simplex virus type 2 meningitis. Clin Infect Dis. 2012;54(9):1304.

Bartleson JD, Swanson JW, Whisnant JP. A migrainous syndrome with cerebrospinal fluid pleocytosis. Neurology. 1981;31(10):1257.

Castels-van Daele M, Standaert L, Boel M, Smeets E, Colaert J, Desmyter J. Basilar migraine and viral meningitis. Lancet. 1981;1(8234):1366.

Caminero AB, Pareja JA, Arpa J, Vivancos F, Palomo F, Coya J. Migrainous syndrome with CSF pleocytosis. SPECT findings. Headache. 1997;37(8):511.

Gómez-Aranda F, Cañadillas F, Martí-MassóJF. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain. 1997;120 ( Pt 7):1105.