An interesting case of Leptomeningitis

1. FRY’DAY NOON CONFERENCE

2. CHIEF COMPLAINT
• Intermittent left upper extremity numbness and left sided facial numbness
10/17/14
UNIVERSITY OF MISSOURI
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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, 4th 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
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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
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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.
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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.
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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
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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
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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
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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.
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11. FLAIR Sequence
Post contrast GRE Sequence
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12. NEUROPHYSIOLOGICAL STUDIES
• EEG:
Normal EEG. Background 9Hz with no ongoing seizure activity or interictal
changes. – Dr. Bandyopadhyay, MD
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13. LUMBAR PUNCTURE
11cm of H2O
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14. RIGHT CAROTID ANGIOGRAM
Cerebral Angiogram is unremarkable for any
Vasculitis or abnormalities of flow.
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15. 10/17/14
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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.
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UNIVERSITY OF MISSOURI
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17. DIFFERENTIAL DIGNOSIS
1. FOCAL CEREBRITIS
2. POSTICTAL PHENOMENON
3. MENINGEAL CARCINAMATOSIS
4. ASEPTIC MENINGITIS
5. CONGENITAL PIAL ABNORMALITY
6. VENOUS THROMBOSIS
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18. WHAT IS NEXT…?
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19. LEPTOMENINGEAL ENHANCEMENT
The differential diagnosis for leptomeningeal enhancement depends on whether it is focal or
diffuse.
Diffuse
• Leptomeningeal carcinomatosis, e.g. from carcinoma of breast or lung, melanoma,
ependymoma
• Hemorrhage, e.g. post-subarachnoid
• Intracranial hypotension, e.g. after lumbar puncture or CSF leak
• Meningitis
• Pyogenic meningitis
• Viral meningitis
• Tuberculous meningitis (can also be focal)
• CNS cryptococcal infection
• Encephalitis
• Granulomatous conditions
• Neurosarcoidosis (can also be focal)
• Post-operative (late finding)
• Post-traumatic (late finding)
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UNIVERSITY OF MISSOURI
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20. CONTINUED…
Focal
• Leptomeningeal carcinomatosis, e.g. from carcinoma of breast or lung,
melanoma
• Hyperemia: post-ictal
• Infarction: subjacent acute (leptomeningeal collaterals) or sub acute
• Lymphoma
• Meningitis (localised), e.g. tuberculous
• Encephalitis
• Neurosarcoidosis
• Scar, postoperative
• Vasculitis
• Neurosyphilis
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UNIVERSITY OF MISSOURI
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21. WHAT IS IVY’S SIGN?
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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
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23. 10/17/14
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