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Unraveling a Complex Neurological Case Study | Diagnosis Insights & Treatment Plan

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This case presentation depicts a 37-year-old Caucasian man with recurrent right facial droop, arm and leg tingling, weakness, and other concerning symptoms. Extensive workup and differential diagnoses were explored, ultimately leading to a diagnosis of Mollaret's Meningitis and Pseudomigraine with Temporary Neurologic Symptoms. The text delves into the patient's history, examination findings, diagnostic tests, treatment modalities, and the unique characteristics of these conditions.

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Unraveling a Complex Neurological Case Study | Diagnosis Insights & Treatment Plan

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  1. Neurology Case Presentation Scott M. Shorten, MD PGY-3

  2. 37 y.o. Right-handed Caucasian man CC: right facial droop, right arm and leg tingling and weakness

  3. 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

  4. PMHx/SurgHx • COPD • Hyperlipidemia • Depression • Septic thrombophlebitis, R Cephalic vein • Appendectomy • Hemorrhoidectomy

  5. Family History • Mother: Bell’s Palsy, Thyroid disease • Father: Meniere’s Disease • Grandmother: Stroke

  6. SocHx • Married, lives in Lawrence • Diesel mechanic • Smokes 1ppd x 30 years • No use of EtOH or Recreational Drugs

  7. Medications • Verapamil 60mg TID • Carbamazepine 200mg BID • Aspirin 325 qD • Famotidine 10mg qD • Trandolapril 2mg qD • Multivitamin • Simvastatin 40mg qHS • Albuterol PRN • Allergy: Minocycline

  8. 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*

  9. Motor: Tone and bulk normal, 5/5 throughout Sensory: decreased pinprick Right UE & LE Reflexes: Coordination: normal F-N-F and Heel-shin Gait: normal x4, no Romberg 2 2 2 2 2 2 3 3 ~ ~ 1 1

  10. ?

  11. 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

  12. Lumbar Punctures: RBCs WBCs ProtGlu 3/7/11 90 20 (88%L) 62 49 3/9/11 2750 15 (51%L) 80 59 3/14/11 140 10 (77%L) 83 60 4/12/11 1 2 70 60 10/3/11 1 2 51 63 5/23/12 550 33 (94%L) 76 60

  13. No growth of bacteria or fungus • CryptococcalAb: 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 agab +Early agab; - Capsid IgM

  14. ??

  15. 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

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

  17. 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 frequency Canadian Medical Assn. http://www.cmaj.ca/content/174/12/1710.2/F2.expansion.html Ginsberg L. PractNeurol 2008;8:348-361 Aurelius E. Clinical Infectious Diseases .2012.

  18. Pseudomigraine with Temporary Neurologic Symptoms and Lymphocytic Pleocytosis = Migrainous Syndrome with CSF Pleocytosis = Syndrome of Transient Headache and Neurologic Deficits with CSF Pleocytosis (HaNDL)

  19. 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

  20. Lumbar Punctures: RBCs WBCs ProtGlu 3/7/11 90 20 (88%L) 62 49 3/9/11 2750 15 (51%L) 80 59 3/14/11 140 10 (77%L) 83 60 4/12/11 1 2 70 60 10/3/11 1 2 51 63 5/23/12 550 33 (94%L) 76 60

  21. Usually in 30s-40s (range 7-52 yrs) • 25-40% had preceding cough/rhinitis/fatigue/diarrhea • No consistent gender predominance

  22. Other Studies • Neuroimaging is usually normal • Leptomeningeal enhancement • Hypoperfusion on CT perfusion • EEG generally shows slowing in the corresponding region

  23. 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

  24. 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

  25. 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

  26. ShalabiM, Whitley RJ. Recurrent benign lymphocytic meningitis. Clinical Infect Dis. 2006;43(9):1194. L Ginsberg, J Kidd. Chronic and Recurrent Meningitis. PractNeurol 2008;8:348-361. Aurelius E, Franzen-Röhl E, GlimåkerM. Long-term valacyclovirsuppressive treatment after herpes simplex virus type 2 meningitis. Clin Infect Dis. 2012;54(9):1304. BartlesonJD, Swanson JW, WhisnantJP. A migrainous syndrome with cerebrospinal fluid pleocytosis. Neurology. 1981;31(10):1257. Castels-van Daele M, Standaert L, Boel M, Smeets E, Colaert J, DesmyterJ. Basilar migraine and viral meningitis. Lancet. 1981;1(8234):1366. CamineroAB, Pareja JA, Arpa J, Vivancos F, Palomo F, CoyaJ. Migrainoussyndrome with CSF pleocytosis. SPECT findings. Headache. 1997;37(8):511. Gómez-Aranda F, Cañadillas F, Martí-MassóJF. Pseudomigrainewith temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain. 1997;120 ( Pt 7):1105.

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