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supranuclear palsy and central pontine myelinolysis, of which pseudobulbar palsy is a frequent manifestation, forced laughing and crying are less dramatic or, in many cases, absent Therefore the pathologic emotional state cannot be equated with pseudobulbar palsy even though the two usually occur together Is this pathologic state, whether one of involuntary laughing or of crying, activated by an appropriate stimulus In other words, does the emotional response accurately re ect the patient s affect or feeling There are no simple answers to these questions One problem, of course, is to determine what constitutes an appropriate stimulus for the patient in question Virtually always, the emotional response is set off by some stimulus or thought; but usually it is tri ing, or at least it appears so to the physician Merely addressing the patient or making some casual remark in his presence may suf ce Certainly the emotional response is out of all proportion to the stimulus Oppenheim and others stated that these patients need not feel sad when crying or mirthful when laughing, and at least in some cases this is in agreement with our experience Other patients, however, do report a congruence of affect and emotional experience Noteworthy also are the stereotyped nature of the initial motor facial response and the relatively undifferentiated nature of the emotional reaction Laughter or crying, as each proceeds, may merge, one with the other Poeck puts great emphasis on the latter point, but it does not seem surprising when one considers the closeness of these two forms of emotional expression a feature that is particularly evident in young children Normal persons may cry when overjoyed and smile when sad More impressive to us is the fact that in some patients with pseudobulbar palsy, laughing and crying, or caricatures thereof, are the only available forms of emotional expression; intermediate phenomena, such as smiling and frowning, are lost In other patients with pseudobulbar palsy, it seems to us, there are lesser degrees of forced laughing and crying, perhaps bridging the gap between this phenomenon and the type of emotional lability discussed earlier Wilson, in his discussion of the anatomic basis and mechanism of forced laughing and crying, pointed out that both involve the same facial, vocal, and respiratory musculature and have similar visceral accompaniments (dilatation of facial vessels, secretion of tears, etc) Two major supranuclear pathways control the pontomedullary mechanisms of facial and other movements required in laughing and crying One is the familiar corticobulbar pathway that runs from the motor cortex through the posterior limb of the internal capsule and controls volitional movements; the other is a more anterior pathway which descends just rostral to the knee of the internal capsule and contains facilitatory and inhibitory bers Unilateral involvement of the anterior pathway leaves the opposite side of the face under volitional control but paretic during laughing, smiling, and crying (emotional facial paralysis); the opposite is observed with a unilateral lesion of the posterior pathway Wilson s argument, based to some extent on clinicopathologic evidence, was that in pseudobulbar palsy it was the descending motor pathways, which naturally inhibit the expression of the emotions, that were interrupted, although he was uncertain of the exact level Almost 40 years later, Poeck, after reviewing all the published pathologic anatomy in 30 veri ed cases, was able to do no more than conclude that supranuclear motor pathways are always involved, with loss of a control mechanism somewhere in the brainstem between thalamus and medulla However, this clinical state is observed in amyotrophic lateral sclerosis, where the corticobulbar tracts may be involved at a cortical and subcortical level As mentioned earlier, the lesions are bilateral in practically all instances (see Poeck in
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the References) There are, however, reports of spasmodic laughter lasting a month or two, following unilateral striatocapsular infarction (Ceccaldi et al), and occasional cases after unilateral pontine infarction or arteriovenous malformation, but these cases have not been veri ed pathologically Of interest is the bene cial effect on distressing pseudobulbar displays of drugs such as imipramine and uoxetine (Schiffer et al) A study has also shown bene t from dextromethorphan combined with quinidine in the pseudobulbar state of ALS (Brooks et al) In a number of personally observed cases, both the emotional lability and pathologic laughter and crying were partially suppressed by these drugs; in others there was no effect Several small series have demonstrated a reduction in the intensity of both forced crying and laughter with the use of antidepression drugs in the majority of patients A rare but probably related syndrome is le fou rire prodromique (prodromal laughing madness) of Fere, in which uncontrol lable laughter begins abruptly and is followed after several hours by hemiplegia We have seen two such cases in which basilar artery occlusion evolved after a brief bout of such forced laughter Martin cites examples where patients laughed themselves to death Again, the pathologic anatomy is unsettled Protracted laughing and (less often) crying may occur also as a manifestation of epileptic seizures, usually of psychomotor type Ictal laughter is usually without affect Daly and Mulder referred to these as gelastic seizures The concurrence of gelastic seizures and precocious puberty is highly characteristic of an underlying hamartoma (or other lesion) of the hypothalamus (see Chaps 16 and 27)
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