Man‑in‑the‑Barrel Syndrome (MIBS)

Man‑in‑the‑Barrel Syndrome (MIBS)


Drunkard’s Cloak by Alice Morse Earle

Definition
B/l UE weakness (brachial diplegia) w/ normal strength in the face, neck, and LE

Etiology
- watershed infarcts or ABI
- NMJ disorders
- b/l brachial neuropathy
- MND
- pontine myelinolysis
- cervical SCI, syringomyelia


MIBS 2/2 Bi-brachial MND

MIBS 2/2 Bi-brachial MND


GCA - Neurology

GCA - Neurology

2022 ACR
Absolute requirement: ≥ 50 yo
Points-based criteria: ≥6 points

Scores
- Southend GCA Probability Score (GCAPS) is sensitive
- Bhavsar‑Khalidi (BK) Score is specific
> does not include age, but theoretically only used for > 50 yo
- HAS‑GCA Score incorporates neuroimaging in GCAPS
> neuroimaging is additive

Treatment
- MTP pulse 250-1000 mg/d for 3 days
If oral, prednisone 60 mg/d
> after remission, taper pred for 20 mg for 3 months, and 5 mg for a year
- start tocilizumab 162 mcg/wk ASAP by ACR

Angelman syndrome

Angelman Syndrome
Dr. Angelman used the term "Happy Puppet Children"; he reportedly conjured this term after seeing the painting "Young Man with a Drawing of a Puppet" at the Castelvecchio Museum in Verona

Genetics
- Maternal deletion, paternal imprinting in 15q11-13
- MCC UBE3A deletion
*minority from paternal uniparental disomy
*Prader-Willi Syndrome is the opposite

Clinical manifestations
- happy demeanor: inappropriate laugh
- stiff/ataxic, tremulous limbs, flap hands when walking
- lack of speech, gesturing for communication
- stereotypies, fascination w/ water-related items & crinkly items
- hypopigmentaion of hair, skin, eyes
- microcephaly, severe ID

Epilepsy
- > 80% of patients
- EEG: high amplitude, rhythmic 2-3 Hz frontally-based activity with intermittent epileptiform discharges
- delta notch

Diagnosis
Methylation analysis with FISH or microarray

Plasmapheresis (PLEX)

Plasmapheresis (PLEX)

Mechanism
- Blood is filtered to remove plasma -> replaced with albumin/donor plasma/saline -> blood cells are returned to the body

'PV = (1 – Hct) × TBV'

'TBV (L) = 0.07 × weight (kg)

- 1 session 1-1.5PV exchange, and removes 63% mediators
- citrate is used as AC, so check calcium

Indications
- MG crisis
- GBS
- NMSOD
- AIE
- MS steroid resistant

Timing
- 3 to 7 sessions every other day (MC protocols)
> less, but will need repeat later
> daily, if protocolated

Protocol
- call IR to place CVA (shiley), hem-onc for PLEX and replacement therapary (BioBank)
- short note
> daily PT, PTT, Fibrinogen, CBC, Calcium levels and metabolic panel 
- common side effects
> tingling in mouth and hands 2/2 calcium
> neck discomfort 2/2 shiley

PD - Management (Tricks)

PD - Management (Tricks)
Clinical Cases

Overnight OFF
- take a pill of crexont or take a pill of sinemet CR at bedtime

Ataxia - General

Ataxia - General
Etiologies
Onset
- acute (ischemia or hemorrhage)
- subacute (Whipple, MS, ADEM, Miller-Fisher/Bickerstaff, GAD, Celiac, VGCC, SLE, hashimoto, sarcoid, tumors, paraneoplastic, wernicke, Vit E def, Vit B12 def, Cu def)
- chronic (congenital or hereditary)?

Exposures
- ASMs, chemo, alcohol, heavy metals
- Recent infections

Idiopathic sporadic genetic 
- SCA6 or Freidrich

Genetic ataxias
- SCA pure cerebellar dysfunction +/- neuropathy, tremor, cognitive changes, myoclonus, pyramidal signs (autosomal dominant)
- Freidrich's (AR): gait and limb ataxia, OPTIC ATROPHY, dysphagia, UMN weakness, peripheral neuropathy, cardiomyopathy, diabetes. Onset usually before 25y
- Wilsons (AR): ataxia, tremor, choreoathetosis, +/- bradykinesia, depression/paranoia/delusions, liver failure
- FXTAS (X-linked recessive): - usually 50y of age with prominent intention tremor, cognitive decline, parkinsonism, autonomic dysfunction, peripheral neuropathy, and proximal muscle weakness with atrophy of MCP and splenium
- Mitochondrial ataxias - exercise intolerance, ptosis, progressive external ophthalmoplegia, myopathy, muscle cramps and pain, cognitive or neuropsychiatric difficulties, and seizures
- MSA/PSP

Investigation

- Ataxia panel Univ Chicago
- Other Labs:
CBC, CMP, serum protein, lipid panel (AOA1 is associated with low serum albumin and elevated LDL), cholestanol (cerebrotendinous xanthomatosis), Vit B12/folate, Vit E, TSH, TPO/TG, RPR, SPEP, A1c, HIV, thiamine, phytanic acid (Refsum's disease), copper/ceruloplasmin, rheumatologic panel, ESR, CRP, celiac panel, alpha-fetoprotein (AT and AOA2), hexosaminidase, and autoimmune encephalitis panel (including GAD65), celiac studies


Management

Ongoing trials

Troriluzole
- SCA 1,2,3,6,7,8,10
- prodrug riluzole; benefits 1xd, not food effect
- FDA reported bias

Early Onset PD

Early Onset PD

Age
EOPD < 50 yo vs LOPD > 50 yo
> YOPD 21-40 yo
> Juvenile parkinsonism < 21yo

Management
- difficult to acheive goals

Tuning Forks

Tuning Forks

Iconographie photographique de la Salpêtrière vol. 2

"If you want to find the secrets of the universe, think in terms of energy, frequency, and vibration"
Nikola Tesla (1856 –1943), Serbian-American engineer

History
1550 Gerolamo Cardano - transmission of sound per skull
1684 Schelhammer - fork to differentiate hearing disorders
1711 John Shore - invented TF
1834 Johann Heinrich Scheibler - different frequencies
1855 - Weber test
1870 - technical improvements

Parts
Prongs (wings), stem (handle), and foot (base)

Motion
- air pressure changes out (compression) & in (rarefaction)
- pure tone (1-dominant frequency) 2/2 simple shape
- fixed frequency (wings length, thickness, material)
> striking hard change amplitude, but not pitch

Classification
- based in frequency: 128, 256, 512, 1024 and higher (research)
> ⬇️ Hz for vibration; ⬆️ Hz for acoustic
> more specific: 128 for vibration and 512 for acoustic

- weighted vs non-weighted
> "Weight feel; no weight hear"
> weight effects: ↓vibration frequency stability; ↑amplitude & duration of vibration; ↑tactile (vibratory) sensation; ↓sound clarity

- quantitative vs non-quantitative
> quantitatives: Rydel‑Seiffer
*vibrameter/biothesiometer - electronic vibration device

Use
- over bone surfaces
- neurological (assess post column) and otological (assess hearing)
- air (indirect) versus bone (direct) conduction

Tests
- Rinne: air vs bone conduction
- Weber: lateralization of sounds

- Schwabach test
> examiner normal hearing
1st place TF on patient's mastoid
2nd patient tells no longer hearing tone
3rd examiner place TF on his own mastoid

- Bing test
> TF placed on mastoid, finger used to alternately open&close ear canal

- ABC (absolute bone conduction)
> Schwabach test with tragus closed, to avoid ambient sound

- Gelle test
> TF placed on mastoid, Siegel speculum for air pressure on TM

Summary

Rydel‑Seiffer
Interesting
- most common clinical TFs are C and its octaves (C3 512Hz; C1 128Hz); in music, standard is A (A4 440Hz)
- A440 (pitch standard, Stuttgart pitch)

- TF, sinusoidal curve, 2 TFs and Lissajous figures

NeuroOncology - Post NSGY

Post-NSGY Care

LP Timing
- Medulloblastoma (always) and ependymoma (depends) both get post‑op LPs — but never immediately after surgery; needs to be done > 14 days after surgery.
> high chance of CSF spread

224. Accepted

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