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Pathological laughter associated with paroxysmal kinesigenic dyskinesia: A rare presentation of acute disseminated encephalomyelitis.

Chaudhry N, Puri V, Patidar Y, Khwaja GA - Epilepsy Behav Case Rep (2012)

Bottom Line: Awareness was retained during the attack, and there was no associated emotional abnormality.It can occur as a result of cerebral lesions like tumors, trauma, vascular insults, multiple sclerosis and/or degenerative disorders.Magnetic Resonance Spectroscopy (MRS) confirmed a demyelinating pathology, and the patient responded well to steroids.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, G.B. Pant Hospital, New Delhi 110002, India.

ABSTRACT
A 13-year-old boy presented with recurrent episodes of sudden brief posturing of the right upper and lower limbs accompanied by transient inability to speak and a tendency to smile which would sometimes break into laughter. Awareness was retained during the attack, and there was no associated emotional abnormality. The events were precipitated by walking and occurred several times in a day. The laughter was pathological in nature, and the abnormal posturing was akin to 'paroxysmal kinesigenic dyskinesia' (PKD). 'Pathological laughter or crying' is defined as an involuntary, inappropriate, unmotivated laughter, crying or both, without any associated mood change. It can occur as a result of cerebral lesions like tumors, trauma, vascular insults, multiple sclerosis and/or degenerative disorders. It can also be a component of gelastic epilepsy which is characterized by stereotyped recurrences, presence of interictal and ictal epileptiform discharges and absence of external precipitants. In our patient, however, there was no ictal or interictal EEG correlate. Paroxysmal kinesigenic dyskinesia is characterized by intermittent, involuntary movements triggered by kinesigenic stimuli and is usually familial but can also be secondary to metabolic and structural brain disorders. Magnetic Resonance Imaging (MRI), in our case, revealed multiple T2 and FLAIR hyperintense, non-enhancing lesions in the periaqueductal gray matter, pontine and midbrain tegmentum, bilateral thalami and left lentiform nucleus suggesting a diagnosis of 'acute disseminated encephalomyelitis', in which this unique combination of pathological laughter and PKD has not been described so far. Magnetic Resonance Spectroscopy (MRS) confirmed a demyelinating pathology, and the patient responded well to steroids.

No MeSH data available.


Related in: MedlinePlus

Magnetic Resonance Imaging (MRI) brain showing T1 hypointense and T2/FLAIR hyperintense lesions in the pontine tegmentum, without any contrast enhancement.
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f0010: Magnetic Resonance Imaging (MRI) brain showing T1 hypointense and T2/FLAIR hyperintense lesions in the pontine tegmentum, without any contrast enhancement.


Pathological laughter associated with paroxysmal kinesigenic dyskinesia: A rare presentation of acute disseminated encephalomyelitis.

Chaudhry N, Puri V, Patidar Y, Khwaja GA - Epilepsy Behav Case Rep (2012)

Magnetic Resonance Imaging (MRI) brain showing T1 hypointense and T2/FLAIR hyperintense lesions in the pontine tegmentum, without any contrast enhancement.
© Copyright Policy - CC BY-NC-SA
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4150596&req=5

f0010: Magnetic Resonance Imaging (MRI) brain showing T1 hypointense and T2/FLAIR hyperintense lesions in the pontine tegmentum, without any contrast enhancement.
Bottom Line: Awareness was retained during the attack, and there was no associated emotional abnormality.It can occur as a result of cerebral lesions like tumors, trauma, vascular insults, multiple sclerosis and/or degenerative disorders.Magnetic Resonance Spectroscopy (MRS) confirmed a demyelinating pathology, and the patient responded well to steroids.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, G.B. Pant Hospital, New Delhi 110002, India.

ABSTRACT
A 13-year-old boy presented with recurrent episodes of sudden brief posturing of the right upper and lower limbs accompanied by transient inability to speak and a tendency to smile which would sometimes break into laughter. Awareness was retained during the attack, and there was no associated emotional abnormality. The events were precipitated by walking and occurred several times in a day. The laughter was pathological in nature, and the abnormal posturing was akin to 'paroxysmal kinesigenic dyskinesia' (PKD). 'Pathological laughter or crying' is defined as an involuntary, inappropriate, unmotivated laughter, crying or both, without any associated mood change. It can occur as a result of cerebral lesions like tumors, trauma, vascular insults, multiple sclerosis and/or degenerative disorders. It can also be a component of gelastic epilepsy which is characterized by stereotyped recurrences, presence of interictal and ictal epileptiform discharges and absence of external precipitants. In our patient, however, there was no ictal or interictal EEG correlate. Paroxysmal kinesigenic dyskinesia is characterized by intermittent, involuntary movements triggered by kinesigenic stimuli and is usually familial but can also be secondary to metabolic and structural brain disorders. Magnetic Resonance Imaging (MRI), in our case, revealed multiple T2 and FLAIR hyperintense, non-enhancing lesions in the periaqueductal gray matter, pontine and midbrain tegmentum, bilateral thalami and left lentiform nucleus suggesting a diagnosis of 'acute disseminated encephalomyelitis', in which this unique combination of pathological laughter and PKD has not been described so far. Magnetic Resonance Spectroscopy (MRS) confirmed a demyelinating pathology, and the patient responded well to steroids.

No MeSH data available.


Related in: MedlinePlus