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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 (MR) spectroscopy through left pontine lesion showing normal NAA peak, mild elevation of choline, and no lactate peak.
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f0030: Magnetic Resonance (MR) spectroscopy through left pontine lesion showing normal NAA peak, mild elevation of choline, and no lactate peak.

Mentions: Routine investigations revealed normal hemoglobin, blood counts, blood sugar, serum electrolytes, and liver and kidney function tests. Serum calcium, phosphorous, alkaline phosphatase and thyroid function tests were normal. X-ray chest was unremarkable. Human immunodeficiency virus types 1 and 2 by ELISA were nonreactive. MRI brain revealed T1 hypointense and T2/FLAIR hyperintense lesions in pontine tegmentum, midbrain tegmentum (left > right), periaqueductal gray matter, bilateral thalamus and left lentiform nucleus, without any contrast enhancement (Figs. 2–5). MR spectroscopy through left pontine lesion showed normal NAA peak, mild elevation of choline, and no lactate peak, suggestive of a benign pathology (Fig. 6). Scalp EEG did not reveal any abnormality. On prolonged video-EEG monitoring the habitual events were recorded without any ictal correlate or any interictal discharges (Video 2).


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 (MR) spectroscopy through left pontine lesion showing normal NAA peak, mild elevation of choline, and no lactate peak.
© Copyright Policy - CC BY-NC-SA
Related In: Results  -  Collection

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

f0030: Magnetic Resonance (MR) spectroscopy through left pontine lesion showing normal NAA peak, mild elevation of choline, and no lactate peak.
Mentions: Routine investigations revealed normal hemoglobin, blood counts, blood sugar, serum electrolytes, and liver and kidney function tests. Serum calcium, phosphorous, alkaline phosphatase and thyroid function tests were normal. X-ray chest was unremarkable. Human immunodeficiency virus types 1 and 2 by ELISA were nonreactive. MRI brain revealed T1 hypointense and T2/FLAIR hyperintense lesions in pontine tegmentum, midbrain tegmentum (left > right), periaqueductal gray matter, bilateral thalamus and left lentiform nucleus, without any contrast enhancement (Figs. 2–5). MR spectroscopy through left pontine lesion showed normal NAA peak, mild elevation of choline, and no lactate peak, suggestive of a benign pathology (Fig. 6). Scalp EEG did not reveal any abnormality. On prolonged video-EEG monitoring the habitual events were recorded without any ictal correlate or any interictal discharges (Video 2).

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