Leptomeningeal Enhancement

An interesting case of Leptomeningitis

1. Dr. Naresh Mullaguri, MD Resident Physician Department of Neurology University of Missouri FRY’DAY NOON CONFERENCE

2. Chief complaint Intermittent left upper extremity numbness and left sided facial numbness 10/17/14 University of Missouri Healthcare

3. HPI 69 y/o right handed white female, came as a direct admit from St. Mary’s hospital for evaluation of the new onset intermittent numbness in her left upper extremity and left side of the face on 09/10/2014 to Neurology Stroke service. Started 10 days ago, 4 th stereotypical episode. Gradually progressed from left forearm to face Sometimes associated with slurring of speech, but not often Usually lasts 2-3 minutes with complete resolution of symptoms. Twice or thrice a day Not associated with HA, dizziness, double vision, nausea or vomiting, dysphagia, dysarthria, bladder or bowel incontinence, fever, chills, weight loss, trauma to the neck or head. De Novo symptoms 10/17/14 University of Missouri Healthcare

4. PERTINENT histories PAST MEDICAL HISTORY: HYPOTHYROIDISM, GLAUCOMA, HYPERLIPIDEMIA, VERTIGO PAST SURGICAL HISTORY: TONSILLECTOMY, TUBECTOMY, D&C SOCIAL HISTORY: JEFFERSON CITY WITH HUSBAND, RETIRED BANKER. OCCASIONALLY DRINKS WINE, DENIED SMOKING AND DRUG USE. FAMILY HISTORY: TIA AND HYPERTENSION. GRANDSON WITH SEIZURE MEDICATIONS: Levothyroxine, Effexor (Hot flashes ), Lovastatin. Aspirin was prescribed recently. 10/17/14 University of Missouri Healthcare

5. Physical Examination Vital Signs: Temp : 36.8 C Pulse : 69 bpm Resp : 16/min BP: 120/72mm of Hg General : Lying comfortably on the bed, talkative and in no distress HENT: No pallor or icterus, moist oral mucosa, No malar rash Neck: supple, normal range of motion with no lymphadenopathy or thyromegaly Cardiovascular: Regular rate and rhythm, no adventitious sounds heard. Respiratory system: non labored breathing, clear to auscultation bilaterally Gastrointestinal: soft, non tender and non distended with no palpable organomegaly. Extremities: no cyanosis, tenderness or effusions in the joints, no purpura or rashes noticed. Psychiatric : Appropriate mood and affect. Very pleasant to talk to. No delusions or hallucinations. 10/17/14 University of Missouri Healthcare

6. Neurological Examination Higher Mental Functions: Alert, awake and oriented X 4, attention and concentration are good, naming, repetition, reading and writing are intact, speech is fluent with no dysarthria, able to perform multistep commands, Memory was intact to recent and remote events. MMSE is 30/30. Cranial Nerve examination: Optic Nerve: visual fields are full to confrontation 3,4 and 6: extra ocular movements were intact to saccades and smooth pursuit movements in both the horizontal and vertical directions. Pupils were round, equal in size and reacting to light bilaterally, No relative afferent pupillary defect. Didn’t perform Fundoscopic exam initially. No nystagmus or double vision. Trigeminal Nerve: Slightly decreased sensation in the left side of face in the V2 distribution to light touch and pinprick, temperature sensation is normal, Medial and lateral Pterygoids strength were normal. Jaw jerk was within normal limits Facial Nerve: No facial asymmetry, normal production of tears Vestibulocochlear: No hearing loss to finger rub bilaterally Glossopharyngeal, Vagus: Strong voice, Uvula is in midline and elevates symmetrically Spinal accessory Nerve: Shoulder shrug is 5/5 bilaterally, SCM were strong bilaterally Hypoglossal: Tongue protrudes to midline with normal movements and no atrophy. 10/17/14 University of Missouri Healthcare

7. EXAM continued… MOTOR SYSTEM EXAM: Bulk and tone were normal in bilateral upper and lower extremities. Strength is 5/5 in bilateral upper and lower extremities. No tremors or abnormal movements observed. Deep tendon reflexes were 3+/4 in bilateral biceps, brachioradialis, triceps, knees. Bilateral ankle jerks are 1+/4. Coordination: Intact to finger to nose test bilaterally, Able to perform rapid alternating movements, no truncal ataxia. SENSORY SYSTEM EXAMINATION: Sensations are intact to light touch and pin prick in all the extremities. Temperature sensation is normal in all the extremities. Plantars are down going bilaterally. Romberg’s sign is negative. 10/17/14 University of Missouri Healthcare

8. CINICAL DIFFERENTAL DIAGNOSIS Transient Ischemic attack with localization to right parietal cortex Vs Brainstem (Vascular Phenomenon) Partial seizure with localization to the Right parietal region (Abnormal electrical phenomenon) Complicated Migraine – atypical (Abnormal Neurochemical phenomenon) Neoplastic/ Autoimmune process in the Right parietal region 10/17/14 University of Missouri Healthcare

9. INVEStigations CBC – white count: 8.3, Hb : 11.7, MCV: 86, PLT: 242 ESR: 22 PT/INR: 13.6/1.0, PTT: 47.5 after Heparin CMP: Na, K, Cl , CO2, Anion gap, BUN and Creatinine were WNL, Glucose and HbA1c – WNL. Total protein – 6.2 Calcium: 9.2 LFTs WNL, Lipid profile – WNL, TSH: 2.69 10/17/14 University of Missouri Healthcare

10. RADIOLOGY Outside CT, non contrast was unremarkable for any acute/sub acute stroke/bleeding. Chest x-ray: Scattered calcified Granulomas MRI of the Brain: An area of abnormal signal intensity in the cortical and subcortical right posterior frontal and anterior parietal region with T2/FLAIR hyperintensities within the sulci with effacement. It shows focal meningeal enhancement of the same region. Scattered non-specific T2/FLAIR subcortical and periventricular hyperintensities. Cystic structure/prominent VR space in the left Hippocampal region. No DWI changes or ICH. Concerns: SAH, Focal cerebritis, Post-ictal changes MRA of the Head and Neck: unremarkable except for a small outpouching of 1.5mm at the intracavernous portion of the left internal carotid artery. 10/17/14 University of Missouri Healthcare

11. FLAIR Sequence Post contrast GRE Sequence 10/17/14 University of Missouri Healthcare

12. Neurophysiological Studies EEG: Normal EEG. Background 9Hz with no ongoing seizure activity or interictal changes. – Dr. Bandyopadhyay, MD 10/17/14 University of Missouri Healthcare

13. Lumbar puncture Protein – 43 Glucose – 54 Lactic acid – 1.4 WBC – 0 RBC – 110 IgG index – WNL Oligoclonal bands – Negative MBP – 1.84 Celiac panel – Negative ACE level – Normal Thyroglobulin ab – normal CSF Crypto – Negative, VDRL - Negative Serum HSV index – Positive West Nile/ St. Louis viral panel – Negative ANA panel – Negative Culture of CSF for fungus, AFB – negative 11cm of H2O 10/17/14 University of Missouri Healthcare

14. Right Carotid Angiogram Cerebral Angiogram is unremarkable for any Vasculitis or abnormalities of flow. 10/17/14 University of Missouri Healthcare

15. 10/17/14 University of Missouri Healthcare

16. RADIOLOGY CONTINUED.. Repeat CT of the head is negative for SAH Repeat MRI showed the same lesions. PAN CT of the Head is negative for any metastatic lesions PET CT is negative for any increased uptake in the Meninges or the underlying cortex Functional MRI : Suboptimal exam but no focal activation was seen in the expected areas – Dr. Ajay Agarwal. 10/17/14 University of Missouri Healthcare

17. DIFFERENTIAL DIGNOSIS FOCAL CEREBRITIS POSTICTAL PHENOMENON MENINGEAL CARCINAMATOSIS ASEPTIC MENINGITIS CONGENITAL PIAL ABNORMALITY VENOUS THROMBOSIS 10/17/14 University of Missouri Healthcare

18. What is Next…? 10/17/14 University of Missouri Healthcare

19. LEPTOMENINGEAL ENHANCEMENT The differential diagnosis for leptomeningeal enhancement depends on whether it is focal or diffuse. Diffuse L eptomeningeal  carcinomatosis, e.g. from carcinoma of breast or lung, melanoma, ependymoma H emorrhage, e.g. post-subarachnoid I ntracranial  hypotension, e.g. after lumbar puncture or CSF leak M eningitis P yogenic meningitis V iral meningitis T uberculous meningitis (can also be focal) CNS cryptococcal infection E ncephalitis G ranulomatous conditions N eurosarcoidosis (can also be focal) P ost -operative (late finding) P ost -traumatic (late finding ) 10/17/14 University of Missouri Healthcare

20. Continued… Focal L eptomeningeal carcinomatosis, e.g. from carcinoma of breast or lung, melanoma H yperemia : post-ictal I nfarction : subjacent acute (leptomeningeal collaterals) or sub acute L ymphoma M eningitis  (localised), e.g. tuberculous E ncephalitis N eurosarcoidosis S car , postoperative V asculitis N eurosyphilis 10/17/14 University of Missouri Healthcare

21. In 1995 Ohta et all.. reported diffuse leptomeningeal enhancement on post contrast T1 weighted MRI in children with Moyamoya disease and named these finding the “ivy-sign”, because it resembles ivy creeping on stones. The ivy-sign can be found on fluid attenuation inversion recovery (FLAIR) images, as well as on contrast enhanced MRI What is IVY’s sign? 10/17/14 University of Missouri Healthcare

22. Moya Moya : On angiography ( conventional or MR ) the characteristic ‘puff of smoke’ ( moya moya in Japanese language) is seen which represent the lenticulostriate and thalamostriate collaterals. Sometimes slow flowing engorged pial vessels and thickened arachnoid membranes give rise to appearance of bright sulci on FLAIR also called the leptomeningeal ‘ivy sign’. Also leptomeningeal enhancement is seen after giving intravenous contrast, also called the contrast enhanced ‘ivy sign’ Educational points 10/17/14 University of Missouri Healthcare

23. My Sincere thanks to Dr. Goyal, Munish, MD Dr. Asher, Irving, MD Dr. Chuquilin Arista, Miguel, MD Dr. Rani, Prityi, MD 10/17/14 University of Missouri Healthcare