Neurology Case Presentation
Scott M. Shorten, MD
PGY-3
37 y.o. Right-handed Caucasian 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 in Lawrence
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: 2 2 2
2 2
2
3
3
~
1
1
~
Coordination: normal F-N-F and Heel-shin
Gait: normal x4, no Romberg
?
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
3/7/11
3/9/11
3/14/11
4/12/11
10/3/11
5/23/12
20 (88%L)
15 (51%L)
10 (77%L)
2
2
33 (94%L)
90
2750
140
1
1
550
Prot
Glu
62
80
83
70
51
76
49
59
60
60
63
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 6/17 but normal on subsequent
9/21: “possible transient due to viral infection”
• EBV studies: +Capsid IgG +Nuclear ag ab +Early
ag ab; - Capsid IgM
??
Mollaret’s Meningitis
v.
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’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
Mollaret, P. Revue Neurologique. 1944 .
Shalabi, M. Clinical Infectious Diseases. 2006.
Pierre Mollaret (1898-1987)
• Most commonly due to HSV-2, often with mucocutaneous lesions found elsewhere
• Diagnosis confirmed with CSF HSV PCR
• Valacyclovir prevented genital lesion recurrence
in first year, but no change in meningitis
frequency
Canadian 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. 1981
Gomez-Aranda, F. Brain. 1997
Lumbar Punctures:
RBCs
WBCs
3/7/11
3/9/11
3/14/11
4/12/11
10/3/11
5/23/12
20 (88%L)
15 (51%L)
10 (77%L)
2
2
33 (94%L)
90
2750
140
1
1
550
Prot
Glu
62
80
83
70
51
76
49
59
60
60
63
60
• Usually in 30s-40s (range 7-52 yrs)
• 25-40% had preceding
cough/rhinitis/fatigue/diarrhea
• No consistent gender predominance
Other Studies
• Neuroimaging is usually normal
– Leptomeningeal enhancement
– Hypoperfusion on CT perfusion
• EEG generally shows slowing in the corresponding
region
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.