Clinical Manifestation

The onset ranges from insidious to abrupt. The syndrome is characterized by a history of constipation (defined as three or more days without bowel movement) followed by a subacute progression of bulbar and extremity weakness (within four to five days) manifest in inability to suck and swallow, weakened voice, ptosis, hypotonia, that may progress to generalized flaccidity and respiratory compromise. There is, however, a broad clinical spectrum of IB. The mild end of the spectrum appears to be represented by infants who never require hospitalization but who have feeding difficulties, mild hypotonia, and floppy neck, and failure to thrive, while the severe end of the spectrum may be characterized by a presentation resembling sudden infant death syndrome (SIDS) (26), and these patients require hospitalization for treatment of their respiratory and feeding difficulties.

The main clinical feature of the syndrome is constipation which occurs in about 95% of patients (16,27). Botulism is expressed clinically as a symmetric, descending paralysis. Early in the progression, weakness, and hypotonia are typical, and the first sign of illness is in the cranial nerves, in the form of bulbar palsies. Less vigorous crying or sucking or subdued facial expression generally is the first sign. Weakness progresses in a symmetric descending fashion over hours to a few days, from muscle innervated by cranial nerves to those of trunk and limbs. A marked dichotomy between the normal physical and abnormal neurologic findings usually occurs.

The time between the onset of constipation and onset of weakness ranges from 0 to 24 days (mean 11 days). Progression is more severe is infants younger than two months (14,28). Obstructive apnea due to the hypotonia leading to collapse of the hypopharynx support can occur rapidly in this age group. The infants also may manifest tachycardia, difficulty in sucking and swallowing, listlessness, weakening, hypotonia, general muscular weakness with a loss of head control, and pooling of oral secretion. These babies appear "floppy," and may manifest various neurologic signs such as ptosis, ophthalmoplegia, sluggish reaction of the pupils, dysphagia, weak gag reflex, and poor anal sphincter tone (29). In seriously ill babies respiratory arrest may occur.

The first signs noted in IB are classically those of autonomic blockade. The parasympa-thetic nervous system is more vulnerable to cholinergic blockade by botulinum toxin than the sympathetic nervous system because the parasympathetic pre- and postsynaptic transmissions are affected. In infants with botulism, recognition of the signs and symptoms associated with parasympathetic blockade is important, since these findings precede generalized motor weakness and respiratory decompensation (17,30). The autonomic nervous system dysfunction may include decreased salivation, distention of abdomen and bladder, decreased bowel sounds, fluctuation in blood pressure, heart rate, and skin color.

The orderly sequence of presentation and recovery of disease signs and symptoms in IB generally follows the order of constipation and tachycardia, followed by loss of head control, difficulty in feeding, weakening, and depressed gag reflex, followed by peripheral motor weakness and subsequent diaphragmatic weakness (30,31).

The nadir of paresis and paralysis generally occur within one or two weeks. The resolution of disease signs and symptoms occurs in the inverse order of presentation, with autonomic finding the last to regress (31). Once strength and tone begin to return, the improvement continues over the following weeks in the absence of complications. It is important to minimize interventions that increase complications.

It is important to remember that at this stage of the disease, return of peripheral motor activity does not signify complete reversed cholinergic synapse. The infant is highly susceptible to events that will additionally stress or impair neuromuscular transmission. Such events may lead to sudden respiratory arrest or gradual respiratory failure. Two specific factors have been associated with respiratory decompensation in IB: administration of aminoglycoside antibiotics and neck flexion during positioning for lumbar puncture or computerized axial tomography scan (32,33). Aminoglycoside antibiotics decrease acetylcholine release from nerve terminals innervating the diaphragm, leading to diaphragmatic weakness and respiratory failure.

Constipation Prescription

Constipation Prescription

Did you ever think feeling angry and irritable could be a symptom of constipation? A horrible fullness and pressing sharp pains against the bladders can’t help but affect your mood. Sometimes you just want everyone to leave you alone and sleep to escape the pain. It is virtually impossible to be constipated and keep a sunny disposition. Follow the steps in this guide to alleviate constipation and lead a happier healthy life.

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