SALIENT FEATURES

HISTORICAL DATA 21 YEAR OLD , FEMALE

BIRTHDAY CELEBRATION IN A FARM

CANNED VEGETABLES

SALIENT FEATURES
SYMPTOMS PROGRESSIVE BLURRING OF VISION

NAUSEA AND VOMITING

DIFFICULTY SPEAKING AND SWALLOWING GENERALIZED BODY WEAKNESS

SALIENT FEATURES
SIGNS

PTOSIS

SALIENT FEATURES

SALIENT FEATURES
SIGNS OPTHALMOPLEGIA (EXTERNAL & INTERNAL)

PTOSIS
FACIAL WEAKNESS DEPRESSED REFLEXES

RESPIRATRY DISTRESS

IS THERE A NEUROLOGIC PROBLEM?

A. SIGNS OF MENINGEAL IRRITATION

NO NUCHAL RIGIDITY B. SIGNS OF INCREASED INTRACRANIAL PRESSURE? (-) HEADACHE (-) DEPRESSION IN THE LEVEL OF CONSCIOUSNESS (-) DIPLOPIA (+) VOMITING

IS THERE A NEUROLOGIC PROBLEM?

C. PRESENCE OF FOCAL NEUROLOGIC DEFICITS

1. WEAKNESS: GENERALIZED DESCENDING TYPE 2. HYPOACTIVE REFLEXES

IS THERE A NEUROLOGIC PROBLEM

WEAKNESS PLUS HYPOACTIVE REFLEXES NEGATIVE BABINSKI

LOWER MOTOR NEURON TYPE OF WEAKNESS

VHC

SPINAL OR PERIPHER AL NERVE

NMJ

MUSCLE
LMN LESION

 BULBAR NMJ INVOLVEMENT

FLUCTUATING NMJ WEAKNESS

ABSENT SENSORY
NMJ MANIFESTATIONS

PRE SYNAPTIC POST SYNAPTIC SYNAPTIC BOTULISM INSECTISID MYASTHEN ES IA GRAVIS LAMBERT SNAKE EATON VENOM MYASTHEN TOXIN IC SYNDROM E

BOTULINUM TOXICITY
ACUTE
PATTERN OF WEAKNESS

FOOD

EXPOSURE

PRESYNAPTIC NMJ
BOTULISM
FACIAL WEAKNESS

LEMS
TRUNCAL AND LIMB

OPHTHALMOPARESIS
SPEECH/ SWALLOWING

WEAKNESS NORMAL PUPILS

IMPAIRMENT DRY MUCOSA IMPAIRED PUPILLARY CONSTRICTION

FOODBORNE BOTULISM
DISEASE MECHANISM: INGESTION OF TOXIN ABSORPTION

STOMACH :NOT ABSORBED; TOXIN COMPLEX (ASSOCIATED PROTEIN) RESISTANT TO PROTEOLYSIS INTESTINE: ALKALINE pH INTESTINE DISSOCIATES TOXIN FROM ASSOCIATED PROTEIN DISSOCIATED TOXIN IS ABSORBED

BOTULINUM TOXIN
COMPLEX ( PROTOXIN + AUXILIARY PROTEIN) CLEAVAGE OF PROTOXIN

1. HEAVY CHAIN A.C TERMINAL - BINDING TO TARGET B. N TERMINAL TRANSLOCATE L CHAIN 2. LIGHT CHAIN ZINC DEPENDENT ENDOPEPTIDASE CLEAVE PROTEINS FORMING SYNAPTIC VESICLE FUSION AND DOCKING COMPLEX

PATH OF TOXIN
1.

PASSAGE OF TOXIN FROM GI TRACT TO VASCULATURE A. ABILITY TO CROSS CELLS- TYPE A&B B. C TERMINUS OF HEAVY CHAIN 2. TOXIN PASSES OUT OF VASCULATURE TO PRESYNAPTIC REGIONS DOES NOT CROSS BBB

PATH OF TOXIN
STEPS IN DEVELOPMENT OF PRESYNAPTIC

BLOCKADE 1. BINDING TO RECEPTORS ON UNMYELINATED PRESYNAPTIC MEMBRANE (DOUBLE RECEPTOR) 2. UPTAKE OF TOXIN INTO NERVE TERMINALS BY ENDOCYTOSIS 3. TRANSLOCATION TO CYTOSOL 4. INHIBITION OF TRANSMITTER EXOCYTOSIS FROM PRESYNAPTIC TERMINAL

PATH OF TOXIN
4.INHIBITION OF TRANSMITTER EXOCYTOSIS BLOCK NT RELEASE AT PERIPHERAL CHOLINERGIC NERVE TERMINALS NEUROMUSCULAR JUNCTION AUTONOMIC NERVE TERMINALS SYMPATHETIC AND PARASYMPATHETIC

PATH OF TOXIN
4.INHIBITION OF TRANSMITTER EXOCYTOSIS BLOCK NT RELEASE AT PERIPHERAL CHOLINERGIC NERVE TERMINALS NEUROMUSCULAR JUNCTION AUTONOMIC NERVE TERMINALS SYMPATHETIC AND PARASYMPATHETIC

BOTULISM DIAGNOSIS
1.

ELECTROPHYSIOLOGICAL STUDY a. REDUCED CMAP AMPLITUDE IN AT LEAST 2 MUSCLES b. AT LEAST 20% FACILITATION OF CMAP AMPLITUDE DURING TETANIC STIMULATION INCREMENTAL RESPONSE c. PERSISTENCE OF FACILITATION FOR AT LEAST 2 MINUTES AFTER ACTIVATION d. NO POSTACTIVATION EXHAUSTION

JOLLY TESTING
HYPOTHENAR

MUSCLES

DECREMENT

INCREMENT

BOTULISM DIAGNOSIS
2. ELECTROMYOGRAM MUSCLE DENERVATION FIBRILLATION AND POSITIVE SHARP WAVES

 FIBRILLATION
POSITIVE SHARP

WAVE

FASCICULATION

BOTULISM DIAGNOSISIMPLICATED 3. ANALYSIS OF SERUM, FECES AND

FOOD MOUSE TOXICITY ASSAY ELISA TEST FOR RAPID SCREENING

END PLATE ELECTROPHYSIOLOGY

MEPP FREQUENCY : REDUCED EPP QUANTAL CONTENT : REDUCED