Lhermitte sign Radiology

Lhermitte's sign - Wikipedi

Combined-System Disease | NEJM

Anatomic-radiologic basis of Lhermitte's sign in multiple

Lhermitte's sign (pronounced Ler-meets) is a sudden sensation resembling an electric shock that passes down the back of your neck and into your spine and may then radiate out into your arms and legs. It is usually triggered by bending your head forward towards your chest Lhermitte's sign (also known as Lhermitte's phenomenon and the barber chair phenomenon) describes a transient sensation of an electric shock extending down the spine and/or extremities upon flexion of the neck, often a sequela of neurologic disease. It was first described by Marie and Chatelin in 1917

PURPOSE: To correlate the magnetic resonance (MR) imaging features of and histologic findings in Lhermitte-Duclos disease (LDD) (dysplastic gangliocytoma). MATERIALS AND METHODS: MR imaging and histologic data from eight patients with the diagnosis of LDD were retrospectively reviewed. Gross pathologic specimens were also available in one case The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). Radiology 1995;194:699-703. Link, Google Scholar; 18 Ortiz O, Bloomfield S, Schochet S. Vascular contrast enhancement in Lhermitte-Duclos disease: case report. Neuroradiology 1995;37:545-548. Crossref, Medline, Google Schola Enroll in our online course: http://bit.ly/PTMSK DOWNLOAD OUR APP: iPhone/iPad: https://goo.gl/eUuF7w Android: https://goo.gl/3NKzJX GET OUR ASSESSMENT B..

Lhermitte's sign is classically associated with multiple sclerosis (MS), a condition characterized by episodes of weakness, sensory changes, and/or visual deficits. In addition to MS, Lhermitte's sign can be the result of many other problems that affect the cervical spinal cord.  In summary, Lhermitte-Duclos disease, or dysplastic gangliocytoma of the cerebellum, is a pathognomonic radiologic entity, the diagnosis of which should remain not only in the bailiwick of neuroimaging specialists but general radiologists as well

SUMMARY: We describe the case of a 26-year-old man who presented with symptoms compatible with Lhermitte sign that occurred during yawning. It was associated with congenital partial aplasia of the posterior arch of the atlas. Cervical multisection-detector CT myelography during yawning showed compression of the upper cervical cord due to the inward mobility of the isolated posterior tubercle Summary: Lhermitte-Duclos disease (LDD) is a rare cerebellar lesion with features of both malformation and benign neoplasm. However, the fundamental nature of the entity, its pathogenesis, and the exact genetic alterations remain unknown Lhermitte's sign is a physical exam maneuver that is considered to have a positive result when a patient experiences an electric-like shooting pain that radiates from the back of the neck down the back when the neck is bent forward by a physician or other health care professional

Lhermitte's sign, also called Lhermitte's phenomenon or the barber chair phenomenon, is often associated with multiple sclerosis (MS). It's a sudden, uncomfortable sensation that travels. Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of the cerebellar cortex. The pathogenesis of the disease is still poorly understood Lhermitte-Duclos disease Radiology Spotter Series-36 By Dr Sumer Sethi About Press Copyright Contact us Creators Advertise Developers Terms Privacy Policy & Safety How YouTube works Test new. Another helpful sign is the Lhermitte sign. This consists of electric shock-like sensations that run down the center of the patient's back and shoot into the limbs during flexion of the neck

Abstract. Lhermitte's sign was experienced by 33.3% of 114 patients with multiple sclerosis, and in 16% it occurred in the first episode. Eleven patients with subacute combined degeneration of the cord due to pernicious anaemia also were reviewed, and one reported Lhermitte's sign There are various appearances called target sign (or bull's eye sign), related to many organs and systems. Even for a single organ, various entities result in a target appearance. For instance, cerebral metastases ( Fig. 27 ) and abscesses can cause the target sign, as well as some hematomas in MRI (in which case it is also called concentric ring sign) Mar 22, 2015 - This is a characteristic MRI imaging of Lhermitte-Duclos disease or dysplastic gangliocytoma of cerebellum. Patient was biopsied and diagnosis confirmed. Mar 22, 2015 Radiology Signs on Twitter Q: Six ring enhancing brain lesions with different cause Synonym: Lhermitte's phenomenon, Barber chair phenomenon Definition of Lhermitte's Sign. Lhermitte's sign (pronounced Ler-meets) is an electric shock-like sensations, spreading along the spine in a cervico-caudal direction and into the arms and legs felt upon active or passive flexion of the neck

Lhermitte Duclos Disease Radiology ll Dysplastic cerebellar gangliocytoma 1. LHERMITTE DUCLOS DISEASE DR.SANTOSH ATREYA PHASE-B RESIDENT DEPARTMENT OF RADIOLOGY & IMAGING 2. INTRODUCTION • Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, is a rare tumour of the cerebellum Radiology Case. 2020 Mar; 14(3):1-6 Neuroradiology: Radiographic Findings of Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in a Woman with Cowden Syndrome: A Case Study and Literature Review Joo et al. J ou rna l of R C as e rts diagnosed with Cowden syndrome, who presented to the hospital wit • Objective. —To test the presumption that Lhermitte's sign in multiple sclerosis is the result of a lesion in the cervical spinal cord. Design. —The radiologic files of 887 patients with multiple sclerosis were reviewed. A detailed questionnaire regarding Lhermitte's sign was sent to 75 patients who had undergone magnetic resonance imaging of the brain and cervical spinal cord

Lhermitte-Duclos disease Radiology Reference Article

Lhermitte-Duclos disease is a rare cerebellar dysplastic gangliocytoma. The most common symptoms include headache, nausea, vomiting, blurred vision, and imbalance Lhermitte's sign or the Lhermitte sign (/ l ɛr ˈ m iː t /), sometimes called the barber chair phenomenon, is an electrical sensation that runs down the back and into the limbs.In many patients, it is elicited by bending the head forward. It can also be evoked when a practitioner pounds on the posterior cervical spine while the neck is flexed; this is caused by involvement of the posterior. Lhermitte's Sign & Organic Brain Syndrome Symptom Checker: Possible causes include Multiple Sclerosis. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search

Lhermitte's sign has been noted as a potential side effect of radiation oncology therapies, specifically as an early delayed radiation injury. These often occur within 4 months of radiation therapy. Medical Oncology. Cisplatin or docetaxel neurotoxicity has been tied to Lhermitte's sign. Prognosi Lhermitte's sign, which occurs between 2 and 6 months after irradiation, manifests as electric shock-like sensation radiating down the spine on flexion movement. This is a transient effect and resolves by itself

None developed chronic progressive radiation myelopathy (CPRM) (maximum spinal dose 21.8-69 Gy); 3.9% (17/437) developed a Lhermitte sign (LS) with a median duration of 6 months (range 1-30 months) and was reversible in all patients The sudden feeling of painless but unpleasant electric shock-like discharges originating in the neck or upper back and spreading down the spine into the limbs on flexion of the head was first described in 1917 by Marie and Chatelin and later by Lhermitte in his seminal paper of 1924.1 It is not a specific symptom but is most commonly encountered in cervical spinal cord demyelination caused by multiple sclerosis.2 3 The sign has been found in many other conditions that cause. Lhermitte's sign (LS) is characterized by electric shock like sensation, spreading along the spine in a cervico-caudal direction and also into both arms and legs, which is felt upon forward flexion of the neck. It is a myelopathy resulting from damage to sensory axons at the dorsal columns of the cervical or thoracic spinal cord and a well-known.

Lhermitte's Sign Developing after IMRT for Head and Neck

A Lhermitte sign is a sign of a dysfunctional dorsal column of the cervical spinal cord. This part of the spinal cord is responsible for sending signals to the brain pertaining to information about a sense of touch, proprioception, and vibration No specific treatment exists for Lhermitte's sign or an electric shock-like sensation that extends down the spine and shoots into the limbs lasting just a few seconds elicited during movement of cervical spine, particularly flexion of the neck. This symptom has been reported in various medical-like cervical spondylosis, multiple sclerosis, vitamin. Lhermitte's sign (neck flexion causes 'electric shock'-type sensation radiating down the spine). [patient.info] The sensory examination may be confounded by the presence of diabetes mellitus and a concurrent peripheral neuropathy. [aafp.org] Specificity is 92%for neurologic and radiologicc signs


Lhermitte's sign MS Trus

Our collection comprises over 200 signs. This is the largest known collection of named animal signs in radiology to date. Radiological examples of most of the signs were collected. To corelate the animal term with the radiological appearance, illustrations of the animal term conforming to the radiological image were created Radiology department of the Medical Centre Haaglanden in the Hague and the Rijnland hospital in Leiderdorp, the Netherlands Publicationdate 2010-10-21 Cerebral venous thrombosis is an important cause of stroke especially in children and young adults

Lhermitte Sign - PubMe

  1. Seeing a doctor. Treatments. Summary. Lhermitte's sign is a short, intense sensation that feels similar to an electric shock passing down the neck and spine and radiating through the trunk and.
  2. Radiological signs are classical and distinctive abnormalities, characteristic of a disease or a group of similar pathologies which can be seen either on a plain radiograph or on a computed tomography (CT) scan. These signs are generally based upon and associated with common objects and patterns that we come across in our routine lives
  3. The authors report four patients who suffered the delayed onset of Lhermitte's sign following head and/or neck trauma with no significant neurological deficits. The average onset from the time of the injury was 2½ months. In all patients, there was a full range of movement of the cervical spine with no tenderness and no neurological deficits
  4. e the clinical, neurophysiological and neuroradiological correlations of Lhermitte's sign in a cohort of MS patients and reassess the relevance of this phenomenon in the clinical history of the disease
  5. Lhermitte's sign is due to the damage MS causes to the nerves, particularly demyelination and hyperexcitability. When the head bends forward toward the chest, an awkward and inappropriate..

Lhermitte sign (spinal cord) | Radiology Reference Article radiopaedia.org. https://radiopaedia.org/articles/lhermitte-sign-spinal-cord. Lhermitte sign or the barber chair phenomenon is an electrical shock sensation running down the spine and into the limb on neck flexion. It suggests compression of the upper cervical spinal cord and/or brainstem Lhermitte's sign [ lār´-mēts´ ] a sensation like an electric shock coursing down the spine when the neck is flexed; a fairly common sign in multiple sclerosis

Lhermitte's is thought to be caused by a lesion or scar tissue (from damaged nerve cells) that are located on the upper cervical spinal cord or lower brainstem Lhermitte's sign, the phenomenon of an electric shock passing down your spine, and/or into your limbs, can be a feature of central nervous system disease. It is typically triggered by bending the neck forward (putting your chin to your chest). It was named after Jean Lhermitte, a French Neurologist, who established its significance in 1924 Lhermitte's sign - also known as barber's chair syndrome or Lhermitte's syndrome - is an electric shock sensation starting in the neck and radiating its way down the back. Although Lhermitte's sign and MS go hand in hand, the condition is actually caused by a variety of health conditions

LHERMITTE'S sign (electric shock-like dysesthesia produced by neck flexion) was first described by Marie and Chatelin in 1917. 1 In 1924, Lhermitte et al described in detail a patient with multiple sclerosis and electric dysesthesias. 2 Since then, Lhermitte's sign has been described in association with tumors of the cervical cord, arachnoiditis, cervical spondylitis, radiation myelitis. Lhermitte?s sign, also known as Barber's Chair Phenomenon, is caused by nerves that are no longer coated with myelin. These damaged nerves respond to the movement of your neck, which causes sensations from your neck to your spine. HEALTHLINE. The main symptom of Lhermitte?s sign is an electric sensation that travels down your neck and back Specific causes for Lhermitte's sign (LS) in cancer patients are spinal cord compression, radiation therapy to the spinal cord, and cisplatin chemotherapy. We observed a transient LS in five of 87 patients treated with more than two cycles of 100 mg/m2 docetaxel (Taxotere). LS developed either concurrently or after the onset of docetaxel-induced sensory neuropathy, and disappeared after the. Lhermitte's sign is a sensation of tingling or electric shocks running down the back and legs on flexion of the neck. Some other actions giving rise to similar sensations are: Neck rotation, arm abduction, coughing ,Yawnin

The striated cerebellum: an MR imaging sign in Lhermitte

  1. Nov 29, 2018 - This is a characteristic MRI imaging of Lhermitte-Duclos disease or dysplastic gangliocytoma of cerebellum. Patient was biopsied and diagnosis confirmed
  2. This sign is often associated with multiple sclerosis and is often known as the Barber Chair Phenomenon. To check for this sign, simply ask the patient to flex their neck. The Lhermitte's Sign is considered present if the patient describes an electric shock-like sensation running down their back or into their limbs. A Chiropractic Online CE.
  3. Synonyms for Lhermitte sign in Free Thesaurus. Antonyms for Lhermitte sign. 190 synonyms for sign: symbol, mark, character, figure, device, representation, logo.

Case 144: Dysplastic Cerebellar Gangliocytoma (Lhermitte

Lhermitte sign: Sudden transient electric-like shocks extending down the spine triggered by flexing the head forward. Due to a disorder such as compression of the cervical spine (the portion of the spinal cord within the neck). One patient who experienced Lhermitte sign wrote that: Looking down to sign a hotel register, I felt a spasm like an electric current move along my back from the base. Coexisting MS and Lhermitte-Duclos Disease Case Report Bria K. Casperson 1, Victor Anaya-Baez 1, Stephen S. Kirzinger 2, Ronald Sattenberg 1, Jens O. Heidenreich 1 * Radiology Case. 2010 Aug; 4(8):1-6 :: DOI: 10.3941/jrcr.v4i8.476 Cite this pape Lhermitte's sign was named after Jacques Jean Lhermitte, the renowned French neurologist and neuropsychiatrist who first characterized it. Lhermitte's sign is not specific to MS. It is a symptom that can occur with any abnormality of the cervical spinal cord, usually involving active inflammation or impingement on a disc Resolution of Lhermitte's sign in multiple sclerosis by treatment with weak electromagnetic fields. Lhermitte's sign, the occurrence of an electrical sensation passing down the back to the legs on flexion of the neck is a common and characteristic feature of multiple sclerosis (MS) which is related to spinal cord lesions affecting the posterior columns and cervical nerve roots

Wrong because Jean Lhermitte was not the first to discover it, and not even the first to properly describe it. Doubly wrong - I still don't understand how this bit stuck - because it's not a sign. Lhermitte征的发病机制被认为是脊髓上升性感觉 传导束髓鞘磷脂的可逆性损害,病变引起轴突对机械性变形异常敏感所致。 Lhermitte征的 鉴别诊断 :脊柱 转移瘤 、颈椎病、 颈椎间盘 突出、多发性硬化、外伤后综合征、 亚急性联合变性 、化疗后、颈髓 放射病 、vitamin B12 deficiency(维生素B12缺乏) An award-winning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, GI, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, hand-out notes, interactive material, most commons lists and pictorial differential diagnose Dec 14, 2016 - This is a characteristic MRI imaging of Lhermitte-Duclos disease or dysplastic gangliocytoma of cerebellum. Patient was biopsied and diagnosis confirmed Thank you for your interest in spreading the word about The BMJ. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail

Lhermitte's Sign Multiple Sclerosis / Myelopathy

  1. Noun []. Lhermitte's sign Electric sensations of the spine and limbs induced by flexing the neck forwardUsage notes []. Modern editors often prefer the non-possessive form of medical eponyms such as Lhermitte sign.; References []. Lhermitte J J, Pollak, Nikolas M: 1924.Les douleurs à type discharge éléctrique consécutives à la flexion cephalique dans la sclérose en plaques
  2. Eye signs in radiology 1. EYE SIGNS IN RADIOLOGY -DR. BHAVANA K. 2. EYE OF THE TIGER SIGN 3. EYE OF THE TIGER SIGN PKAN (Pantothenate kinase- associated neurodegeneration) Hallervorden-Spatz syndrome The eye corresponds to the low signal globus pallidus with the central focus of high signal
  3. A sign in chest radiology refers to a radiographic and/or CT scan finding that implies a specific pathologic process. Understanding the meaning of a sign indicates comprehension of an important concept related to the radiologic findings. Knowing the name of the sign is not as important as recognizing and understanding the meaning of the radiologic findings, but it will help in communicating.
  4. Bilateral Babinski's Reflex & Lhermitte's Sign Symptom Checker: Possible causes include Primary Progressive Multiple Sclerosis. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search

Lhermitte's Sign: Symptoms, Causes, Treatmen

Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum is a rare disorder that can cause progressive mass effects to the structures occupying posterior fossa. Magnetic resonance imaging is a diagnostic modality of choice demonstrating characteristic non-enhancing gyriform pattern with the enlargement of cerebellar folia, hypointense on T1 and hyperintense on T2 weighted. Lhermitte sign Neurology Flexion of the neck evokes abrupt spasms over the body, a finding typical of multiple sclerosis as well as cervical spine diseas Lhermitte's sign was described by Marie and Chatelin and named after Jean Lhermitte. This sign is mostly described as an electric shock like condition by some patients of multiple sclerosis. This sensation occurs when the neck is moved in a wrong way or rather flexed. It can also travel down to the spine, arms, and legs, and sometimes the trunk

Acute presentation of Lhermitte-Duclos - Applied Radiolog

Also known as focal hepatic hot spot sign due to its appearance on technetium 99m sulphur colloid scans. Pathophysiology : SVC obstruction -> if partial -> drainage by collateral vessels -> internal thoracic, superficial thoraco-abdominal veins and paraumbilical veins -> left portal vein-> Early arterial enhancement in the QUADRATE lobe of liver -> Hot Quadrate lobe sign on early arterial CT. Lhermitte's sign, also known as the barber's shop sign, is more properly a symptom than a sign, and is described by patients as an electric-like sensation induced by forward flexion of the head. 3,4 It was originally described by Marie and Chatelin in 1917, and one year later by Babinski and Dubois Diagnosis: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) by MRI features. Differential diagnosis of this case includes: cerebellar infarction: excluded by DWI acute cerebellitis: excluded by clinical picture rhombencephalosyn.. Lhermitte's sign is a sudden unpleasant sensation that radiates from the top of the neck and down the spine. It is considered to be a neuropathic pain flare up triggered by certain movements of the head and neck. Individuals who have experienced Lhermitte's sign often describe it as an electric shock or a strong vibration Jean Lhermitte, a French neurologist and neuropsychiatrist, did not publish his first report until 1920. However, in 1924 he did publish the seminal article on the subject which resulted in it becoming well known. Given that Lhermitte's sign is named for Lhermitte, it is incorrect to spell the term as Lhermitte's sign

Lhermitte Sign during Yawning Associated with Congenital

Lhermitte-Duclos disease (LDD; dysplastic cerebellar gangliocytoma) is a rare hamartomatous lesion of the cerebellar cortex and this was first described in 1920. LDD is considered to be part of the autosomal-dominant phacomatosis and cancer syndrome Cowden disease (CS). We examined the brain of a 46-year-old man, who displayed the manifestations of CS, with 7 Tesla (T) and 1.5T MRI and 1.5T MR. Lhermitte's sign, sometimes called the Barber Chair phenomenon, is an electrical sensation that runs down the back and into the limbs.In many patients, it is elicited by bending the head forward[1]. It can also be evoked when a practitioner pounds on the posterior cervical spine while the neck is flexed; this is caused by involvement of the posterior columns

Lhermitte-Duclos Disease: Assessment with MR Imaging

From free‐text clinical narratives (e.g., outpatient encounters, radiology reports, discharge summaries), we extracted patient‐level counts of all clinical terms mapped to concept unique identifiers (CUIs) Lhermitte's sign, and flare were positively associated with relapse,. Lhermitte sign= electric sensation on neck flexion; Interferon beta balances the expression of pro- and anti-inflammatory agents in the brain, and reduces the number of inflammatory cells that cross the blood brain barrier.[4] Overall, therapy with interferon beta leads to a reduction of neuron inflammation.[4 Animal signs in radiology. Introduction. In this article we will provide an educational review of radiological signs, that they are named after and/or having similar appearance to Animals. Each radiological sign will be accompanied by a description of the sign/disease procss involved

What Is Lhermitte's Sign? (with pictures) - Info Bloo

Looking for the definition of LHERMITTE SIGN? Find out what is the full meaning of LHERMITTE SIGN on Abbreviations.com! The Web's largest and most authoritative acronyms and abbreviations resource We've got 0 anagrams for Lhermitte sign » Any good anagrams for Lhermitte sign? This page list all the various possible anagrams for the sentence Lhermitte sign.Use it for solving word puzzles, scrambles and for writing poetry, lyrics for your song or coming up with rap verses Lhermitte‑Duclos disease (LDD) is a rare, non‑cancerous entity characterized by enlarged, abnormally developed cerebellar folia containing dysplastic cells. Symptomatic LDD is commonly observed in adults (adult‑onset LDD, aLDD) as an isolated condition or associated with Cowden's disease (CD) Department of Radiology and Department of Pediatrics, Suleyman Demirel University School of Medicine, Isparta, Turkey Yesildag˘ A, Baykal B, Ayata A, Kerman G, Koroglu M, Olgar S, Oyar O. Lhermitte-Duclos disease associated with neurofibromatosis type-1 and non-ossifying fibroma. Acta Radiol 2005;46:97-100 The PTEN hamartoma tumor syndromes (PHTS) are a collection of rare clinical syndromes characterized by germline mutations of the tumor suppressor PTEN. These syndromes are driven by cellular.

Lhermitte-Duclos disease | Image | Radiopaedia

A middle-aged or young adult presenting with Lhermitte's sign should undergo a very careful neurologic examination to rule out possible MS. (Lhermitte's sign is present if a patient complains of paresthesia or electric shock-like sensation which radiates down long the length of the spine or up through the head upon flexing the neck. Lhermitte-Duclos disease (LDD) (English: /ˌlɛərˈmiːtˌduːˈkloʊ/), also called dysplastic gangliocytoma of the cerebellum, is a rare, slowly growing tumor of the cerebellum, a gangliocytoma sometimes considered to be a hamartoma, characterized by diffuse hypertrophy of the granular layer of the cerebellum. It is often associated with Cowden syndrome. It was described by Jacques Jean. Lhermitte-Duclos Disease and Vascular Malformation 1461-1472, 1999 8) Meltzer CC, Smirniotopoulos JG, Jones RV: The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease. Radiology 194: 699-703, 1995 9) Moonis G, Ibrahim M, Melhem ER: Diffusion-weighted MRI in Lhermitte-Duclos disease: report of two cases

Optic neuritis

Purpose: Lhermitte's phenomenon (LP) is a rare manifestation, which is defined when a sudden electric-shock sensation transmitted down the spine induced by neck flexion; however, the reverse LP is defined when symptoms are induced by neck extension, not flexion. Because reports of LP are limited in the Taiwan literature, we report this case Lhermitte's sign (par-esthesias spreading down the spine, often into the legs, on neck movement) is typical for a demyelinating lesion of the cervical posterior columns, but can be, although rarely, seen in other conditions that involve the same site. Other characteristic syndromes include isolate J Neuropathol Exp 17. Meltzer CC, Smirniotopoulos JG, Jones RV (1995) The striated Neurol 61:215-229 cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dys- 3. Robinson S, Cohen AR (2006) Cowden disease and Lhermitte- plastic gangliocytoma). Radiology 194:699-703 Duclos disease: an update. Case report and review of the. On physical examination upper motor neuron signs are present including hyperreflexia, clonus, spasticity, Lhermitte's phenomenon, up-going plantar response, and Hoffmann's sign . The incidence and prevalence of myelopathy due to degeneration of the spine are estimated at a minimum of 41 and 605 per million in North America, respectively Lhermitte-Duclos disease is a rare disorder characterized by a slowly enlarging mass lesion in the cerebellum. The pathophysiology of the disease is p We use cookies to enhance your experience on our website.By continuing to use our website, you are agreeing to our use of cookies

Lhermitte-Duclos disease (LDD) is a rare hamartomatous focal or diffuse enlargement of the cerebellum with unclear etiology. The characteristic magnetic resonance imaging (MRI) appearance consists. This patient went on to have a posterior fossa craniotomy and biopsy, confiming the diagnosis of Lhermitte-Duclos disease. Further investigations for possible Cowden syndrome did not demonstrate any hamartomatous lesions or tumors elsewhere Because the mucocutaneous features may develop several decades after birth, the present case both supports the previously reported association between Lhermitte-Duclos disease and Cowden's syndrome and highlights the need for long-term follow-up monitoring of a pediatric patient with Lhermitte-Duclos disease because of the risk of malignancies associated with Cowden's syndrome

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