A case of manganese induced parkinsonism in hereditary haemorrhagic telangiectasia

Yoshikawa K; Matsumoto M; Hamanaka M; Nakagawa M; Yoshikawa, K; Matsumoto, M; Hamanaka, M; Nakagawa, M
September 2003
Journal of Neurology, Neurosurgery & Psychiatry;Sep2003, Vol. 74 Issue 9, p1312
Academic Journal
journal article
A 44 year old right handed woman complained of difficulty in moving. She and her relatives had skin telangiectasia or recurrent epistaxis. On neurological examination, she had a mask-like facies and bradykinesia in both extremities. Laboratory examinations showed iron deficiency anaemia and mild liver dysfunction with raised serum manganese. On T1 weighted cranial magnetic resonance imaging there were hyperintense areas in the globus pallidus bilaterally, suggesting manganese deposition. Abdominal angiography confirmed multiple portal-systemic shunts in the liver, and a needle biopsy of the liver showed diffuse dilatation of the sinusoids with fatty change. Levodopa did not improve the bradykinesia. This appears to be a case of hereditary haemorrhagic telangiectasia with manganese induced parkinsonism, which may be a new type of neurological disorder in such patients.


Related Articles

  • Technique modifications for septodermoplasty: an illustrative case. Bastianelli, Mark; Kilty, Shaun J. // Journal of Otolaryngology -- Head & Neck Surgery;12/30/2015, Vol. 44, p1 

    Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that results in telangiectasia of the sinonasal tract, gastro-intestinal tract as well as possible arteriovenous malformations of the lung, liver and brain. One of the most common disease manifestations of...

  • Epistaxis in hereditary hemorrhagic telangiectasia: an evidence based review of surgical management. Chin, Christopher J.; Rotenberg, Brian W.; Witterick, Ian J. // Journal of Otolaryngology -- Head & Neck Surgery;1/12/2016, Vol. 45, p1 

    Patients with Hereditary Hemorrhagic Telangiectasia (HHT) frequently present with epistaxis. Up to 98 % of these patients will have epistaxis at some point in their life. There are multiple ways to deal with this problem, including conservative, medical and surgical options. We present a case...

  • Acute stroke-like presentation of acquired hepatocerebral degeneration. Smita, B.; Gafoor, V. Abdul; Saifudheen, K.; Jose, James // Annals of Indian Academy of Neurology;Apr2014, Vol. 17 Issue 2, p204 

    Neurological manifestations in liver diseases have been well-described. Parkinsonism developing in cirrhotic patients is a unique clinical, neuroradiological, and biological entity. The symptoms are often insidious in onset and occur after liver disease has made its presentation. Acute...

  • Optimal management of hereditary hemorrhagic telangiectasia. Garg, Neetika; Khunger, Monica; Gupta, Arjun; Kumar, Nilay // Journal of Blood Medicine;2014, Vol. 5, p191 

    Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is a group of related disorders inherited in an autosomal dominant fashion and characterized by the development of arteriovenous malformations (AVM) in the skin, mucous membranes, and/or internal...

  • Bevacizumab: an option for refractory epistaxis in hereditary haemorrhagic telangiectasia. Amann, Arno; Steiner, Normann; Gunsilius, Eberhard // Wiener Klinische Wochenschrift;Aug2015, Vol. 127 Issue 15/16, p631 

    Background: Recurrent epistaxis in hereditary haemorrhagic telangiectasia (HHT) patients significantly decreases their quality of life. Treatment in therapy refractory patients is limited although various options have been tested so far. Case report: Herein, one patient is described that was...

  • REVIEW. Parkinson's disease: chameleons and mimics. Ali, Khalid; Morris, Huw R. // Practical Neurology;Feb2015, Vol. 15 Issue 1, p14 

    Parkinson's disease (PD) is a common neurodegenerative condition that usually presents with symptoms related to asymmetric bradykinesia, resting tremor, rigidity and postural instability. Making the correct diagnosis can be challenging as many conditions—including tremor, gait and...

  • Synthetic dural graft septoplasty in epistaxis from hereditary hemorrhagic telangiectasia. Burckhardt, Wilfred B.; Guerra, Claudia Patricia // Colombia Medica;Jul-Sep2013, Vol. 44 Issue 3, p189 

    It is an autosomal dominant vascular disorder, which has a variety of clinical manifestations, with epistaxis being one of the most common. Many treatment options exist for epistaxis, but with no consensus on which is the method of choice. We describe the case of a patient with hereditary...

  • First experiences with an individual nasal olive in patients with hereditary haemorrhagic telangiectasia (HHT). Al Kadah, Basel; Papaspyrou, George; Schneider, Mathias; Schick, Bernhard // European Archives of Oto-Rhino-Laryngology;Jan2015, Vol. 272 Issue 1, p117 

    Hereditary haemorrhagic teleangiectasia (HHT) is most notably characterized by vulnerable vascular formations of the nasal superficial mucosa. Epistaxis is one of the most common symptoms of the afflicted patients, with an incidence of more than 90 %. A variable series of treatments have been...

  • Minimally invasive surgical techniques for hemostasis in Osler-Weber-Rendu disease. Tuşaliu, Mihail; Zainea, Viorel; Goanţă, Cristina-Maria; Sorică, Andreea // Polish Annals of Medicine / Rocznik Medyczny;Jun2015, Vol. 22 Issue 1, p50 

    Introduction: Osler-Weber-Rendu disease, also called ''hereditary hemorrhagic telangiectasia'' (HHT), is an autosomal dominant genetic disorder characterized by the appearance of small disseminated vascular malformations in the skin, mucous membranes and organs (lungs, brain, liver) with...

  • Additional Extra-striatal Site of Neurodegeneration in Parkinson's Disease. Aktham I. Alemam; Osama F. Obeid // Egyptian Journal of Neurology, Psychiatry & Neurosurgery;Jul2013, Vol. 50 Issue 3, p221 

    Background: Over the past decade, there is an increasing realization that the thalamus is an extra-striatal region of non-dopaminergic degeneration in Parkinson's Disease (PD). Objective: To search for any changes in the thalamic shape or volume in PD by non-invasive structural MRI images....


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics