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. 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. 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. Social History
Former smoker
Used to drink wine
Frequent trips to Guatemala and New Mexico
Family History
Brother had history of hairy cell leukemia
Father with history of lymphoma
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. 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
19. 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
20. Clinical Findings in Patients with
IHCP *
* Goyal et al. Neuroradiology (1997) 39: 619–623
32. 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
33. 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
37. 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
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)