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1. Case Presentation Chief complaint: Jerking
HPI: 67 y.o. A.A. male, 3 d. prior to admission awoke with oscillating, rapid, intermittent, jerking movements in his right hand and leg.
2. PMH: CHF, RNV on 1/99 showed EF 23%
CAD
CRI
Type II DM
HTN
h/o Bladder Outlet Obstruction
GERD
Anemia
3. Medications ECASA
Furosemide 80 bid
CaCO3 1.25 tid
FeSO4 325 qd
Isosorbide dinitrate 20 tid
Glyburide 2.5 qd
4. Uremic Encephalopathy Tim Johnston
November 5, 1999
5. Epidemiology Usually in association with untreated renal failure
Also associated with dialyzed patients
More severe and rapidly progressive in those with acute deteriorations in renal fxn.
Presence correlates poorly with numerical measures of uremia
6. Pathophysiology UNKNOWN
? decreased cerebral blood flow or metabolism?
? electrolyte imbalance--autopsy studies most remarkable for much increased (2x) Calcium levels
7. Pathophysiology, cont. Toxin accumulation inhibits synaptic transmission via GABA in test tubes
? N-terminal PTH? Vit D treatment and parathyroidectomy have normalized EEGs (most evidence seems to support this as a major factor)
8. Clinical Characteristics VARIABILITY: over both the short and long term
Mild sx: Fatigue / apathy, clumsiness, impaired concentration, ?myoclonus? Moderate sx: Emotional lability, frontal lobe dysfunction
Severe sx: Delirium, hallucinations, mid-& high freq. hearing loss, agitation, stupor, coma, seizures.
9. What is Myoclonus? Characteristics:
Rapid, brief, irregular
Usually multifocal and asymmetric
Can be:
spontaneous at rest
induced by stimuli
assoc. with voluntary movements
10. M-M-M-More M-M-Myoclonus Clonus differs in that it is rhythmic and monophasic
Types of myoclonus
Segmental: restricted group of muscles
Multiplex: widespread, lightning-like
11. What causes myoclonus? Metabolic disorders: resp. failure, CRI, hepatic failure, electrolyte disturbance
Encephalitis
Epilepsy (juvenile myoclonic epilepsy) Posthypoxic intention myoclonus
Creutzfeldt-Jacob
Lipid storage disorders
12. Severe Encephalopathy Seizures usually T-C, but can be focal
Common neuro. signs: hyperreflexia, frontal release signs, asterixis, meningism
Tremor, increased tone
Asymmetry common: may even have hemiparesis, which can be alternating
13. Laboratory Variable level of azotemia
CT or MRI may show atrophy; no good studies of MRI findings in uremic encephalopathy
CSF often abnormal; studies before routine dialysis:
In one series, 12% of those with BUN >75mg/dL had >5 nucs / mm3 and 58% with prot >100mg/dL
14. Laboratory, cont. EEG: Diffuse slowing!
percentage of slow waves on the EEG has been proposed as a way to follow adequacy of dialysis
15. Treatment Renal Replacement
16. Other Neurologic Complications in Pts. with Renal Failure Dialysis disequilibrium syndrome
Chronic encephalopathy
Myopathy Uremic neuropathy
Dialysis dementia
Subdural hematoma
Wernickes Encephalopathy
Gangrenous calcification
17. Dialysis Disequilibrium Syndrome Non-specific symptoms: nausea, headache, restlessness up to confusion and seizures
Acute onset
Starting dialysis in profoundly uremic pt. ??? Due to brain swelling from lag in osmolar shifts ???
Self-limited
18. Chronic encephalopathy Even dialyzed patients sometimes aint quite right
Sluggishness, sleep disturbances
Memory / Judgment impaired
Neuropsychological testing shows increased reaction time, problems with perception and learning in CAPD, HD patients alike
19. Uremic Neuropathy Present in the majority of patients with renal failure severe enough to require dialysis or transplantation
Affects axons--??Ca++; ??PTH
Distal, symmetric neuropathy- sensory +/- motor, and ascending
Restless legs; reflex loss
Cured with transplantation; better with HD
20. References Adams, Raymond. 1997. Principles of Neurology (6th ed.). McGraw-Hill, pp. 102-105, 722-728.
Burn, DJ and Bates, D. Neurology and the Kidney. J Neurol Neurosurg Psychiatry 1998; 65:810-821.
Fauci, et al. 1998. Principles of Internal Medicine. McGraw-Hill, p.114.
Lockwood, Alan H. Neurologic Complications of Renal Disease. Neurologic Clinics 1989; 7(3): 617-627.
Moe, S.M. and Sprague, S.M. Uremic encephalopathy. Clinical Nephrology 1994; 42(4):251-256.