2/14/2014 • 55yo male with a history of cryptogenic strokes at age 45 • Chief complaint: left leg swelling, progressive Clinical Pathological Conference multifocal numbness and weakness Cheryl Jay, MD, Professor of Neurology, UCSF Director of Neurology Clinics, San Francisco General Hospital Andrew W. Bollen, MD, DVM Professor of Pathology, UCSF Mark Burish MD, PhD Neurology Resident, UCSF • • Jan 2012: progressive left leg swelling and erythema starting Jan 2012: progressive left leg swelling and erythema starting in the calf and extending to the foot, initially intermittent in the calf and extending to the foot, initially intermittent then became constant and slightly painful then became constant and slightly painful • • March 2012: admitted to an outside hospital for leg swelling March 2012: admitted to an outside hospital for leg swelling and pain. and pain. – Lower extremity Doppler U/S negative – Lower extremity Doppler U/S negative – – Noted to have leukopenia with ANC of 1000 Noted to have leukopenia with ANC of 1000 – – Muscle biopsy with “focal mild endomysial inflammation in 1 Muscle biopsy with “focal mild endomysial inflammation in 1 muscle fascicle with histiocytes” muscle fascicle with histiocytes” – – Treated for possible vasculitis with prednisone, mild improvement Treated for possible vasculitis with prednisone, mild improvement in symptoms in symptoms • • May 15 2012: Feeling of being off balance, needing to hold May 15 2012: Feeling of being off balance, needing to hold on to objects while walking progressing over 1 week to left on to objects while walking progressing over 1 week to left foot drop foot drop • • May 29 2012: Numbness on right medial palm and ring and May 29 2012: Numbness on right medial palm and ring and pinky fingers pinky fingers • June 5 2012: softening of his voice • June 5 2012: softening of his voice • • June 8 2012: Admitted to UCSF June 8 2012: Admitted to UCSF 1
2/14/2014 • • Jan 2012: progressive left leg swelling and erythema starting Jan 2012: progressive left leg swelling and erythema starting in the calf and extending to the foot, initially intermittent in the calf and extending to the foot, initially intermittent then became constant and slightly painful then became constant and slightly painful • • March 2012: admitted to an outside hospital for leg swelling March 2012: admitted to an outside hospital for leg swelling and pain. and pain. – – Lower extremity Doppler U/S negative Lower extremity Doppler U/S negative – – Noted to have leukopenia with ANC of 1000 Noted to have leukopenia with ANC of 1000 – – Muscle biopsy with “focal mild endomysial inflammation in 1 Muscle biopsy with “focal mild endomysial inflammation in 1 muscle fascicle with histiocytes” muscle fascicle with histiocytes” – Treated for possible vasculitis with prednisone, mild improvement – Treated for possible vasculitis with prednisone, mild improvement in symptoms in symptoms • • May 15 2012: Feeling of being off balance, needing to hold May 15 2012: Feeling of being off balance, needing to hold on to objects while walking progressing over 1 week to left on to objects while walking progressing over 1 week to left foot drop foot drop • • May 29 2012: Numbness on right medial palm and ring and May 29 2012: Numbness on right medial palm and ring and pinky fingers pinky fingers • • June 5 2012: softening of his voice June 5 2012: softening of his voice • • June 8 2012: Admitted to UCSF June 8 2012: Admitted to UCSF • • Jan 2012: progressive left leg swelling and erythema starting Past medical history: cryptogenic strokes at age 45 (frontal and cerebellar) in the calf and extending to the foot, initially intermittent – Normal transthoracic and transesophageal echocardiograms then became constant and slightly painful – Holter negative – Normal cerebral angiogram – CSF: 7 WBC (94%L), 8 RBC, glucose 57, and total protein 81, normal IgG index • March 2012: admitted to an outside hospital for leg swelling and pain. • Allergies: None – Lower extremity Doppler U/S negative – Noted to have leukopenia with ANC of 1000 – Muscle biopsy with “focal mild endomysial inflammation in 1 • Medications: Ibuprofen prn muscle fascicle with histiocytes” – Treated for possible vasculitis with prednisone, mild improvement • Family history in symptoms – Daughter with AML at age 16 – • Maternal grandmother with first stroke at age 45, additional strokes in the following years, May 15 2012: Feeling of being off balance, needing to hold died in 50s on to objects while walking progressing over 1 week to left – Father with prostate cancer foot drop – No family history of clotting disorders (DVT, miscarriage, etc) • May 29 2012: Numbness on right medial palm and ring and • Social history pinky fingers – Never smoker, occasional EtOH, no illicits – Operates motorcycle shop. Prior to this illness physically active, running marathons, gardening • June 5 2012: softening of his voice – Travel: Originally born in the San Francisco Bay Area. Has traveled extensively in US. Last trip outside US in 2006 (Spain), has also been to Italy, Japan, China and New Zealand but last was to Spain in 2006. • June 8 2012: Admitted to UCSF – Exposures: Has 2 healthy adult dogs. Pricked by rose thorns in November 2011. Swims in fresh water lakes but not since last summer. Does not eat raw meat or unpasteurized cheese. No known TB contacts, has never been incarcerated or spent time in homeless shelters. 2
2/14/2014 May 6, 2003 May 6, 2003 June 5, 2003 June 5, 2003 • • Past medical history: cryptogenic strokes at age 45 (frontal and cerebellar) Initial general exam – Normal transthoracic and transesophageal echocardiograms – HEENT: normal. Neck supple – Holter negative – CV: normal – Normal cerebral angiogram – Pulm: normal – CSF: 7 WBC (94%L), 8 RBC, glucose 57, and total protein 81, normal IgG index – Abdominal: normal – Skin: Left lower extremity below knee with decreased hair, erythematous • Allergies: None – Musculoskeletal: left calf with 1+ nonpitting edema, tender to palpation • Medications: Ibuprofen prn • Initial neurologic exam • Family history – MS: AOx3, 6 digits forwards, intact naming and repetition, 0/3 recall at 5 – Daughter with AML at age 16 minutes – Maternal grandmother with first stroke at age 45, additional strokes in the following years, – CN: Visual fields full, PERRL, EOMI, facial sensation intact. R lower facial died in 50s droop. Palate midline. Shoulder shrug full. Tongue midline – Father with prostate cancer – Str: No pronator drift, slow right finger taps. Unable to tap with left foot – No family history of clotting disorders (DVT, miscarriage, etc) • Bilateral upper extremities and right lower extremity: full strength • Left lower extremity: Ililopsoas 4+, Quad 4, Hams 4, AnkleFlex 1, AnkleExt 1, • Social history AnkleEvert 1, Ankle Inv 1, Toe flex 1, Toe ext 1 – Never smoker, occasional EtOH, no illicits – Sens: Decreased sensation to pin in the hand (right 5 th digit), otherwise – Operates motorcycle shop. Prior to this illness physically active, running marathons, gardening intact to light touch, pin, temperature, vibration, and proprioception – Travel: Originally born in the San Francisco Bay Area. Has traveled extensively in US. Last trip – Reflexes (R/L): 2+ on right, 3+ on left throughout . Plantar flexor on right, outside US in 2006 (Spain), has also been to Italy, Japan, China and New Zealand but last was to Spain in 2006. extensor on left – Exposures: Has 2 adult dogs, both healthy. Pricked by rose thorns at some point in November – Coord: Normal finger-to-nose bilaterally 2011. Swims in fresh water lakes but not since last summer. Does not eat raw meat or – Gait: Steppage gait on left. Romberg positive. unpasteurized cheese. No known TB contacts, has never been incarcerated or spent time in homeless shelters. 3
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2/14/2014 FLAIR T1 POST-GAD FLAIR T1 POST-GAD • Vascular work-up – Normal intracranial MRA and MRV – Normal Transthoracic and Transesophageal Echos with no shunt – Transcranial dopplers: no emboli detected of the B/L MCAs over 30 minutes • EEG (20 hours) – diffuse slowing and disorganization, worse over the right hemisphere – No epileptiform activity or electrographic seizures • EMG – Normal Motor Nerve Conduction Studies (R ulnar, R median nerves) – Normal Sensory Nerve Conduction Studies (R ulnar, R median, B/L superficial peroneal, B/L sural nerves) – Normal EMG of RUE 5
2/14/2014 MRI leg • Enhancement – left soleus – Left tibialis posterior (post. aspect) – Left gastroc. (medial&lateral heads) • Atrophy and fatty infiltration – left soleus (medial aspect) • Fluid collection – fascial plane b/w soleus & gastroc. • Initial biopsy March 2012 – “focal mild endomysial inflammation in 1 muscle fascicle with histiocytes” 6
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