11/7/2017 Managing Common Neurologic Case #1: Inpatient Problems A Difficult Diagnosis • A 70M presents to the ED with increasing cough and SOB and is intubated for increased work of breathing • A RML infiltrate is seen on CXR • Further history suggests “failure to thrive” S. Andrew Josephson MD with increasing difficulties walking and Carmen Castro Franceschi and Gladyne K. Mitchell Neurohospitalist Distinguished Professor swallowing over the past 5 months Chair, Department of Neurology Director, Neurohospitalist Program University of California, San Francisco The speaker has no disclosures Neurological Examination Neurological Examination (con’t) • Why perform a neurologic examination in • Sensory: Normal this setting? • Reflexes: 2+ in RUE, 3+ in LUE, Patellars • MS: Normal 3+ B, absent at ankles. • CN: Mild bifacial weakness (difficult) • Motor: Atrophy of intrinsic hand muscles. Moderate weakness in all four limbs diffusely. Minimal fasciculations seen 1
11/7/2017 UMN LMN An underutilized cheap test that can be lifesaving Pyramidal Variable Pattern of Weakness • The admitting physician, prior to intubation, asked Function/Dexterity Slow alternate motion rate Impairment of function is RT to come to bedside to measure a Forced Vital mostly due to weakness Capacity (FVC) and Maximal Inspiratory Force Tone Increased Decreased (MIF) – FVC 1.2L, MIF -20 Tendon Reflex Increased Decreased, absent or normal • Check in all patients with suspected neuromuscular weakness including all with weakness of all four limbs Other signs Babinski sign, other CNS signs Atrophy (except with problem (e.g. aphasia, visual field cut) of neuromuscular junction) • Caveats: seal, participation Amyotrophic Lateral Sclerosis Inpatient Management Issues • Combination of UMN and LMN signs on exam • 1. Recognition and making diagnosis – Fasciculations not specific for ALS • 2. Prognosis • Bulbar musculature including respiratory and – Majority die within 5 years swallowing often involved – 10-20% survive longer than 10 years – Poor pulmonary toilet and aspiration: PNA • For established patients…. • Rare: 1-3/100,000; only 10% familial – 1. Ventilatory failure risk: Frequent MIF/FVC – 2. Higher risk of PNA and aspiration: Consider PEG early • Many patients have delayed diagnosis – 3. Disease modifying meds: Riluzole minimally effective • Diagnosis can be made clinically but EMG/NCS is – 4. Multidisciplinary care team is key gold standard – Allows for rare mimics to be addressed as well 2
11/7/2017 Additional (Bigger) Organ Donation: Ventilation Issues It Comes Up • Nighttime BiPap often advised • Hot topic in ethics currently – Screen for morning headaches, confusion, increased • Multiple case reports and series have been symptoms published – Baseline PFTs • Consider non-invasive ventilation first • Important discussion regarding trach – Only around 10% choose trach • Placement issues: resources needs to be at home are great – Communication issues for discussion and GOCs • Most cognitively normal*** Toossi S et al. Ann Neurol 71:154, 2012 Case #2 Case #2 • Exam • A 63yo man comes to the ED with 3 days of inability to walk. The patient reports a 2 – General exam nl with stable vitals – Mental status, cranial nerves normal week history of tingling in his hands and – Motor exam with mild-moderate symmetric feet while also stating that he has been weakness prox>distal in the upper ext., stumbling while walking for five days. distal>prox in the LEs – Sensory exam completely normal – Reflexes 1 throughout except 0 ankles, plantar response flexor bilaterally 3
11/7/2017 Guillain Barre Syndrome: Case #2: Additional Tests Key Points • Clinically must think in the setting of paresthesias and weakness FVC/MIF: 1.1L, -30 – Normal sensory exam, weakness not always ascending – Areflexia the rule, but not early in the disease Lumbar Puncture: Opening pressure normal, 2 – High protein with no cells on LP the rule, but not early WBC, Zero RBC, Protein 87, Glucose normal in the disease • EMG/NCS for diagnosis – Axonal and Demyelinating forms • Antecedent illness or infection only 30% • Other Variants: Miller Fisher variant w/ GQ1b Ab Guillain Barre Syndrome: Case #3 Key Points • What will kill the patient • A 40 yo man comes to the ED with – Respiratory Failure: Intubate for less than 20cc/kg increasing weakness and dyspnea. The • Frequent MIF/FVC patient states that he has a history of • ICU or stepdown care always – DVT/PE: SQ heparin myasthenia gravis diagnosed at an OSH two – Autonomic instability: cardiac (telemetry), ileus weeks ago but “things are going downhill.” • Treatment He is on Mestinon (pyridostigmine) 60mg – IVIg or Pheresis, NOT steroids PO q4hrs and Prednisone 60mg PO qd. MIF – The earlier the better is –10, FVC 250cc 4
11/7/2017 Myasthenic Crisis Myasthenia Gravis: Key Points • True crisis vs. cholinergic crisis • Two types of myasthenia – Young F>M • Triggers – Old M=F – Infection, surgery, initial steroids • Clinical: Bifacial weakness, Ptosis, • Management fluctuations, proximal weakness, respiratory – Usually stop all anti-cholinesterase meds • Diagnosis – Pheresis or IVIg – Antibodies (~90% in generalized myasthenia) – ICU, intubation, DVT/PE prophylaxis – EMG with repetitive stimulation, sfEMG Myasthenia Gravis: Key Points Case #4 • You are called to see a 76M with • Outpatient Management Parkinson’s disease on the orthopedic – Pyridostigmine (Mestinon) service who reports dramatic worsening of – Immunosuppression his disease following an elective hip • Prednisone first then Imuran/CellCept/Cytoxan replacement. – What about the Thymus? • On examination he has a soft voice and can barely move his limbs. He has a mild resting tremor of the LUE 5
11/7/2017 Parkinson’s disease ET PD Speed 5-10Hz 4-6 Hz • Idiopathic form caused by loss of cells in substantial nigra of brainstem Symmetry Symmetric Asymmetric Most Common • Cardinal features Postural Rest Component – 1. Tremor No Yes Helped by EtOH – 2. Rigidity Common Rare – 3. Bradykinesia FH? Autosomal Dominant – 4. Postural instability Any sign of Parkinsonism in an ET pt should lead to questioning the diagnosis Admitted with Falls: Parkinson’s Treatment What the Neurologist Finds 1. Give L-Dopa • 1. Parkinson’s disease - Levodopa/Carbidopa – Exam: tremor, rigidity, postural instability 2. Dopamine Receptor Agonists • 2. Neuropathy -Pramipexole, Ropinirole 3. Alter Dopamine/L-Dopa Metabolism – Exam: numbness, decreased ankle reflexes -MAO-B Inhibitors: selegeline, rasagiline • 3. Cervical Myelopathy -COMT Inhibitors: entacapone – Exam: spasticity, Lhermitte’s sign, increased 4. Decrease Cholinergic tone (rarely used) ankle reflexes 5. Surgery: Deep Brain Stimulation (DBS) 6
11/7/2017 Worsening of PD in House: Worsening of PD in House: Old Medication Issues New Medication Issues • All PD meds are (basically) oral • Avoid all dopamine-depleting medications in these patients if possible • Missing doses for some brittle patients can be incredibly problematic and lead to symptoms – Antipsychotics – Many common antiemetics including prochlorperazine • NPO arrangements are particularly challenging and metoclopramide • Need to write for exact times of medications (not • Consider these medications as culprits in new- bid/tid/etc) onset cases that arise in the hospital • Ask if typically takes medications with food and – Often more symmetric examination try to accommodate Worsening of PD in House: Worsening of PD in House: Concurrent Illness Wrong Diagnosis • Not well described in the literature but commonly • Drug-induced parkinsonism seen in practice • Vascular parkinsonism (not common) • Treating underlying illness key • Parkinson’s plus syndromes – Unlikely “rapid progression” – Progressive Supranuclear Palsy (PSP) • May have to increase medications while sick • Vertical gaze problems • Frequent falls • Be particularly attentive to swallowing – Multiple Systems Atrophy (MSA) issues/aspiration risk and falls while worse • Autonomic instability • Particularly susceptible to delirium as with all • Early ventilation problems and sleep-disordered breathing neurodegenerative disorders 7
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