Unusual presentation of hypothalamic hamartoma with hypersomnia in an adult patient

Tezer, F. İrsel; Pektezel, Mehmet Yasir; Göçmen, Rahşan; Saygı, Serap

doi:10.1684/epd.2014.0669

Cited by 8 publications

(6 citation statements)

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“…Often the sessile masses associate epilepsy and the pedunculated ones may be related to endocrinological disturbances and/or to visual impairment (Kameyama, Masuda, & Murakami, 2010). Some authors suggest that larger hamartomas are frequently associated with higher seizure severity, cognitive impairment, and behavioral disorder as signs of EE probably due to hypothalamic-thalamic-mammillary connections dysfunction (Deonna & Ziegler, 2000;Frattali et al, 2001;Prigatano, 2007;Tezer, Pektezel, Gocmen, & Saygi, 2014). In our experience, the most severe cases with pronounced cognitive decline were the ones that started earlier with GS, suggesting that age of onset may also play a predictor role in HH-ES outcome.…”

Section: Discussionmentioning

confidence: 99%

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

et al. 2019

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IntroductionClinical manifestations of the hypothalamic hamartoma‐epilepsy syndrome (HH‐ES) in adulthood are variable. Efficacy of therapeutic options and outcome are diverse.MethodsRetrospective study of adult patients diagnosed with a HH in magnetic resonance imaging and epilepsy who attended our tertiary Epilepsy Unit between 2003 and 2018. We report the clinical and electroencephalographic features of a series of adult patients with HH and related epilepsy seen in our center together with the treatments and seizure outcome.ResultsWe describe a series of eight patients. Five males (62.5%), median age at evaluation was 28.5 years (IQR: 15.5). Clinical manifestations included focal with preserved and impaired awareness emotional seizures (gelastic seizures [GS]) in six patients (75%), focal tonic, atonic with impaired awareness and focal to bilateral tonic–clonic seizures. Mild GS were the only symptom in one patient. Three patients (37.5%) had endocrinological disturbances such as obesity and hypothyroidism. Fifty percent of the patients showed psychiatric comorbidity such as anxiety disorder and aggressiveness, and two patients had psychomotor delay. Seven patients (87.7%) had drug‐resistant seizures and three of them were treated with radiosurgery. Out of the treated group, only one (33.3%) became seizure‐free 2 years after surgery but developed psychiatric problems. The other two patients had an Engel IV outcome and received a vagal nerve stimulation (VNS) implant. VNS did not lead to changes either in intensity nor in seizure frequency.ConclusionsHypothalamic hamartoma‐epilepsy syndrome clinical manifestations in adult patients are as variable as at pediatric age. Outcome of therapeutic options such as radiosurgery or VNS may be poorer at this stage.

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Section: Discussionmentioning

confidence: 99%

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

et al. 2019

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“…A case study ascribed narcolepsy to damage of the ventral ascending RAS in a patient with a traumatic brain injury [ 5 ]. Many studies have reported the hypothalamus is associated with hypersomnia; thus, the recovery of the patient's hypersomnia was due to the recovery of an injured lower ascending RAS [ 6 ]. As introduced by Moruzzi and Magoun in 1949, the basis of arousal in the brainstem has been linked to the ascending RAS [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Ischemic Stroke of Midbrain and Cerebellum Involving Reticular Activating System

Sadiq

Subhan

2017

Cureus

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“…Many studies have reported close association of the hypothalamus with hypersomnia; thus it appeared that the recovery of this patient's hypersomnia was attributed to the recovery of an injured lower ARAS, particularly the recovery of an injured right ventral lower ARAS which appeared to be most severely injured on 2-month DTT. 5 , 14 , 15 …”

Section: Discussionmentioning

confidence: 99%

“… 1 Many previous studies have suggested that involvement of the ascending reticular activating system (ARAS) might be a pathogenetic mechanism of hypersomnia in stroke patients. 2 – 5 …”

Section: Introductionmentioning

confidence: 99%

Recovery of Hypersomnia Concurrent With Recovery of an Injured Ascending Reticular Activating System in a Stroke Patient

et al. 2016

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We report on a stroke patient who showed recovery of hypersomnia concurrent with the recovery of an injured ascending reticular activating system (ARAS), which was demonstrated by diffusion tensor tractography (DTT).A 70-year-old female patient underwent coiling of the left ruptured posterior communicating artery after subarachnoid hemorrhage and both extraventricular drainage for management of an intraventricular hemorrhage. At 2 months after onset, when she started rehabilitation, she exhibited intact consciousness, with the full score on the Glasgow Coma Scale: 15. However, she showed severe hypersomnia: she always fell asleep without external stimulation and the Epworth Sleepiness Scale (EPS) score was 24 (full score: 24, cut off for hypersomnia: 10). She underwent comprehensive rehabilitative therapy, including neurotropic drugs, physical therapy, and occupational therapy. Her hypersomnia has shown improvement as 14 (3 months after onset), 11 (4 months after onset), 7 (12 months after onset), and 6 (24 months after onset), respectively.On 2-month DTT, narrowing of both lower dorsal and ventral ARASs was observed on both sides: in particular, among 4 neural tracts of the lower ARAS, the right lower ventral ARAS was the narrowest. By contrast, on 24-month DTT, the 4 narrowed neural tracts of both lower dorsal and ventral ARASs were thickened compared with those of 2-month DTT.Recovery of hypersomnia with recovery of an injured lower ARAS on DTT was observed in a stroke patient. Our results suggest that evaluation of the lower ARAS using DTT might be useful for stroke patients with hypersomnia.

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Unusual presentation of hypothalamic hamartoma with hypersomnia in an adult patient

Cited by 8 publications

References 21 publications

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

Ischemic Stroke of Midbrain and Cerebellum Involving Reticular Activating System

Recovery of Hypersomnia Concurrent With Recovery of an Injured Ascending Reticular Activating System in a Stroke Patient

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