Pachymeningitis

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Case presentation during grand rounds. Discussion about possible differential diagnosis and management

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  • Spread of malignant cells through the CSF space. These cells can originated both in primary CNS tumours, as well as from distant tumours that have metastasized
  • Left to rightLeptomeningeal enhancement of chronic inflammation in Tuberculous meningitis. (T1 contrast)Leptomeningealcarcinomatous from breast cancer (T1 contrast)Neurosarcoidosis and Chiari I. (T1 contrast)Meningiomas
  • Intracranial hypotension secondary to CSF overshunting. T1 contrast coronal-axialSpontaneous intracranial hypotension (Small ventricles, slight herniation of tonsils, droopy splenium)
  • Pachymeningitis

    1. 1. Grand rounds Neurology Dept.Daniel Vela-Duarte, MDPGY-2. Department of NeurologyLoyola University Medical CenterMarch 8, 2013
    2. 2. Chief ComplaintTransient speech difficulties and confusion
    3. 3. History of Present Illness 65 years-old left handed man presented to the ED with:  Transient speech difficulties and confusion  Symptoms lasted for 15 minutes. Right sided headaches associated with bilateral tinnitus for three months  Followed an episode of acute sinusitis  Tylenol and Ibruprofen provided minor relief  No photophobia or sonophobia  No jaw claudication, paresthesias or visual disturbances  ESR was 88 Unilateral temporal artery biopsy done April 2012 for presumed GCA:  Negative biopsy  Prednisone 60mg daily
    4. 4. History of Present Illness cont’d Headaches continued  Decreased energy  Increased appetite  Mood changes  Memory difficulties  Hoarseness  Persistent elevation of inflammatory markers (ESR and CRP) Treatment  Prednisone 40mg BID  Methotrexate 4 tabs weekly  Alendronic acid 35mg weekly
    5. 5. Past Medical History Hypothyroidism Right Rotator cuff tear Left knee osteoarthritis No history of hypertension, diabetes, coronary artery disease, hyperlipidemia, strokes, TIAs No history of migraines No history of autoimmune disease
    6. 6. Social History Former smoker Used to drink wine Frequent trips to Guatemala and New MexicoFamily History Brother had history of hairy cell leukemia Father with history of lymphoma
    7. 7. Physical Examination Vitals  BP 128/61 | Pulse 72 | Temp 98.5 °F (36.9 °C) | Resp 18 | SpO2 98% General exam:  Redness, flushed faced.  No tenderness to palpation of Sup. Tem Art. Neurological exam  Higher functions intact Visual acuity stable since 2008. Fundoscopic examination:  Mild hemorrhagic retinopathy  Increased cup/disc ratio  Normal intraocular pressure Inability to fully abduct right eye Remainder of the neurologic examination was normal
    8. 8. Ancillary Data 134 96 10 14 191 15.1 269 3.3 25 1.08 97.3 EEG normal CT chest/abd/pelvis:  Low density area along the posterior wall of the right atrium, suspicious for thrombus. TEE: No evidence of mass or thrombus in the right atrium Galium Scan: Negative for malignancy or sarcoidosis
    9. 9. MRI Brain T1 Post
    10. 10. MRI Brain T1 Post
    11. 11. Differential diagnosis ?
    12. 12. Ancillary tests. SPEP: Hypogammaglobulinemia UPEP: Unable to quantitate globulins Quantiferon gold test for TB: Negative ACE level: Normal ANCA, ANA, ENA, pANCA: Negative Cultures:  Bacterial cultures negative  Fungal cultures negative  HSV negative  HIV negative  RPR negative CRP: 3.8
    13. 13. CSF Studies. 9/26/2012 9/26/2012 RBC 0 /UL 214 (H) 1360 (H)Lumbar puncture Opening pressure WBC 0 - 8 /UL 2 1  26 cm H20 CSF Flow cytometry SEG % 45 53  Limited by small number of cells LYMPH % 41 30 CSF culture MONO % 14 17  Negative Cryptococcal antigen GLUCOSE, CSF 62  Negative PROTEIN 114 (H) APPEARANCE CLEAR
    14. 14. 8 CRP7 6.9 7.3 6.6100 6 Sed rate (mm/hr) 5 5.5 4.6 90 4 3.8 88 2.5 3 2.5 2 80 1.6 1.8 1 0.7 0 70 66 60 50 40 39 29 28 28 30 28 20 22 16 10 11 2 0 4/4/2012 5/4/2012 6/4/2012 7/4/2012 8/4/2012 9/4/2012 10/4/2012 11/4/2012 12/4/2012
    15. 15. Management Increased intracranial pressure  Acetazolamide 250 mg twice daily Prednisone 50mg daily Methotrexate 20mg once a week Pachymeningeal biopsy
    16. 16. Pachymeningeal Biopsy Right temporal biopsy Multinucleated giant cells Chronic inflammatory changes  Increased kappa and lambda plasma cells  Focal acute necrotizing areas containing neutrophils CD20, CD3, CD5 lymphocytes  Negative for pankeratin, CK20 and PSA  Focal CK7 positive staining of myofibroblasts Microscopically negative for microorganisms  PAS, Gram, FITE, AFB Mycobacteria DNA negative.
    17. 17. Differential Diagnosis Idiopathic hypertrophic pachymeningitis IgG4-related disease IgG4-sclerosing pachymeningitis ? Rheumatoid pachymeningitis ? Neurosarcoidosis
    18. 18. Idiopathic hypertrophic pachymeningitis First description in 1869 by Charcot and JoVroy  Association with neurosyhpillis or tuberculosis NaVziger in 1949 described first case of idiopathic hypertrophic cranial pachymeningitis (IHCP) Clinical symptoms results with compression of anatomical structures by thickened meninges *  Headache ~ 88%  Cranial nerve palsy ~ 62 %  Ataxia ~ 32 % Ancillary Investigations:  ESR elevated in up to 41 % patients **  CSF with increased proteins in up to 51% patients *** Parney et al. Neurosurgery (1997) 41:965–971** Kupersmith et al. Neurology (2004) 62:686–694
    19. 19. Clinical Findings in Patients with IHCP ** Goyal et al. Neuroradiology (1997) 39: 619–623
    20. 20. Imaging in Patients with IHCP ** Yu Chan Lee et al. AJNR (2003) 24:119–123
    21. 21. Clinical Outcomes in Patients with IHCP * * Kupersmith et al. Neurology (2004) ;62:686–694
    22. 22. Pachymengitis - Etiology Infective  Neurosyphilis  CNS tuberculosis : tuberculous pachymeningitis  CNS cryptococcosis Chronic bacterial meningitis Inflammatory  Wegeners granulomatosis  Polyarteritis nodosa  Rheumatoid pachymeningitis Neurosarcoidosis Haemodialysis Mucopolysaccharidoses Meningeal metastases, including CNS lymphoma Multiple meningiomas Intracranial involment with Erdheim-Chester disease HTLV-1 Infection
    23. 23. Leptomeningeal EnhancementDiffuse Focal  Leptomeningeal carcinomatosis Leptomeningeal carcinomatosis  Hyperaemia : post-ictal Ependymoma  Infarction : Intracranial Haemorrhage  Leptomeningeal collaterals (SAH)  Lymphoma Intracranial hypotension  Meningitis (localized) Meningitis  Tuberculous Encephalitis  Encephalitis Granulomatous conditions  Neurosarcoidosis Neurosarcoidosis  Postoperative Post-operative (late finding)  Vasculitis Post-traumatic (late finding)  Neurosyphilis
    24. 24. Leptomeningeal carcinomatosis Primary intracerebral malignancies  Glioblastoma multiforme (GBM) and anaplastic astrocytoma  Medulloblastoma  sPNET  Ependymoma  Germinoma  Choroid plexus carcinoma Widespread metastatic disease (more common)  Breast cancer : most common  Lung cancer : most common  Melanoma  Lymphoma and leukemia
    25. 25. IgG4-related disease Mass-forming disorder with frequent systemic involvement  commonly in the pancreas, salivary glands and lacrimal glands. Recently defined disease entity, characterized by a high serum IgG4 concentration and various complications, including:  Mikulicz’s disease  Autoimmune pancreatitis (AIP)  Riedel’sthyroiditis  Sclerosing cholangitis  Retroperitoneal fibrosis  Tubulointerstital nephritis  Hilar lymphadenopathy,  Pseudotumour  Interstitial pneumonia
    26. 26. IgG4-relatedDisease
    27. 27. Five Cases of IgG4-related Disease withHypertrophic Pachymeningitis
    28. 28. Radiological differential diagnosis Images provided by: Bruno Di Muzio, MD and Frank Gaillard et al.
    29. 29. Radiological differential diagnosis
    30. 30. Back to our patient ...
    31. 31.  Memmory difficulties  Short-term memory Incoordination  Left upper extremity ataxia Intermittent binocular horizontal diplopia Episodic confusion and combativeness  EEG: slowing of the normal basic frequency  Excess bilateral 4-7 Hz > 1-3 Hz slowing Depression  Sertraline 50mg /daily  Poor response to Medical management of depression  ECT
    32. 32.  Tocilizumab. (IL-6 receptor blocker - Actemra)  4mg/kg per infusion intravenously once every 4 weeks at least 1 year.  First dose: Feb 04  Second dose Feb 26 with continuation of prednisone 10mg for a month  Treatment interrupted due to increase LFTs Other option considered  Rituxan  Azathoprine (Imuran)  Equivalent to Methrotexate  Loger time to achieve immunosupresion
    33. 33. Response to treatment ?
    34. 34. Response to treatment ?T1, sagital coronal, pre
    35. 35. Idiopathic hypertrophic pachymeningitis. 50% to 66% of patients do no respond to therapy with steroids Other immunosuppressive agent to be considered No clear guidelines for treatment No literature on infliximab and hypertrophic pachymeningitis
    36. 36. Questions ?

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