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Прогрессирующий надъядерный паралич
Другие названияСиндром Стила-Ричардсона-Ольшевского, лобно-височная деменция с паркинсонизмом
СпециальностьНеврология
СимптомыНарушение равновесия, замедленные движения , затрудненное движение глаз , слабоумие
Обычное начало60–70 лет
ПричиныНеизвестный
Дифференциальная диагностикаБолезнь Паркинсона , кортикобазальная дегенерация , FTDP-17 , болезнь Альцгеймера
УходМедикаменты, физиотерапия, трудотерапия
МедикаментЛеводопа , амантадин
Прогноз7–10 лет после постановки диагноза.
Частота6 на 100 000

Прогрессирующий супрануклеарный паралич ( ПСП ) - это дегенеративное заболевание с поздним началом, сопровождающееся постепенным ухудшением состояния и гибелью определенных объемов головного мозга . [1] [2] Состояние приводит к симптомам, включая потерю равновесия , замедление движений , затрудненное движение глаз и когнитивные нарушения. [1] PSP можно ошибочно принять за другие нейродегенеративные заболевания, такие как болезнь Паркинсона , лобно-височная деменция и болезнь Альцгеймера . Причина состояния неизвестна, но связана с накоплением тау-белка.внутри мозга. В некоторых случаях могут быть полезны такие лекарства, как леводопа и амантадин . [1]

PSP затрагивает около шести человек на 100 000 человек. [1] Первые симптомы обычно возникают у людей в возрасте 60–70 лет. Мужчины подвержены заболеванию немного чаще, чем женщины. [1] Не было обнаружено никакой связи между PSP и какой-либо конкретной расой, местом или родом занятий. [1]

Признаки и симптомы [ править ]

Первыми симптомами в двух третях случаев являются потеря равновесия, выпад вперед при мобилизации, быстрая ходьба, натыкание на предметы или людей и падения. [3] [ необходима цитата ] Симптомы деменции также первоначально наблюдаются примерно в каждом пятом случае лобно-височной деменции. [4]

Другими распространенными ранними симптомами являются изменения личности, общее замедление движений и визуальные симптомы. Наиболее частые поведенческие симптомы у пациентов с PSP включают апатию, расторможенность, тревогу и дисфорию. [4]

Более поздние симптомы и признаки могут включать, но не обязательно, деменцию (обычно включающую потерю торможения и способности систематизировать информацию), невнятную речь , затрудненное глотание и затруднение движения глазами , особенно в вертикальном направлении. Последнее является причиной некоторых падений, которые испытывают эти пациенты, поскольку им трудно смотреть вверх или вниз. [5]

Среди других признаков - нарушение функции век , контрактура лицевых мышц , наклон головы назад с ригидностью мышц шеи , нарушение сна , недержание мочи и запор . [5] Некоторые пациенты полностью сохраняют когнитивные функции до конца.

Визуальные симптомы имеют особое значение в диагностике этого расстройства. Пациенты обычно жалуются на трудности с чтением из-за невозможности хорошо смотреть вниз. Примечательно, что офтальмопарез, испытываемый этими пациентами, в основном связан с произвольным движением глаз и неспособностью делать вертикальные саккады , что часто ухудшается при нисходящих саккадах. Пациенты, как правило, испытывают затруднения при взгляде вниз ( паралич опущенного взгляда ), за которым следует паралич взгляда вверх. Этот парез вертикального взгляда будет исправлен, когда экзаменатор пассивно поворачивает голову пациента вверх и вниз в рамках теста на окулоцефалический рефлекс . Непроизвольное движение глаз, вызванное феноменом Белла, например, может быть ближе к норме. При внимательном осмотре могут быть заметны движения глаз, называемые «прямоугольными рывками», когда пациент фиксируется на расстоянии. Это прекрасные движения, которые можно принять за нистагм , за исключением того, что они имеют саккадический характер и не имеют плавной фазы. Хотя здоровые люди также совершают прямоугольные рывки, пациенты с PSP совершают более медленные прямоугольные рывки с меньшими вертикальными компонентами. [6] Оценка этих прямоугольных рывков и уменьшения вертикальных саккад особенно полезна для диагностики прогрессирующего надъядерного паралича, потому что эти движения отличают пациентов с PSP от других пациентов с паркинсонизмом. [6] Трудности с конвергенцией(недостаточность конвергенции), когда глаза сближаются при фокусировке на чем-то близком, например, на страницах книги, является типичным. Поскольку глаза не сводятся вместе для фокусировки на коротком расстоянии, пациент может жаловаться на диплопию (двоение в глазах) при чтении. [5]

Характерная внешность лица, известная как «признак процеруса», с широко раскрытыми глазами, морщинистым лбом с хмурым выражением лица и углублением других лицевых складок является диагностическим признаком PSP. [7]

Signs and Symptoms • Early onset gait and balance problems • Clumsy, slow, or shuffling gait • Lack of coordination • Slowed or absent balance reactions and postural instability • Frequent falls • Slowed movements • Rigidity (generally axial) • Vertical gaze palsy • Loss of downward gaze is usually first • Abnormal eyelid control • Decreased blinking with “staring” look • Blepharospasms (involuntary eyelid spasms) • Double vision • Dystonia, commonly at neck and hands into flexion, but can also be into extension at neck • Speech and swallowing changes • Subcortical dementia (personality changes, slowness of thought) • “Rocket sign,” when patient jumps up quickly from seated position, often falling back in chair

Cause[edit]

The cause of PSP is unknown. Fewer than 1% of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17 (rs1800547), has been linked to PSP.[8] Nearly all people with PSP received a copy of that variant from each parent, but this is true of about two-thirds of the general population. Therefore, the H1 haplotype appears to be necessary but not sufficient to cause PSP. Other genes, as well as environmental toxins, are being investigated as other possible contributors to the cause of PSP.[citation needed]

Additionally, the H2 haplotype, combined with vascular dysfunction, seems to be a factor of progressive supranuclear palsy.[9]

Besides tauopathy, mitochondrial dysfunction seems to be a factor involved in PSP. Especially, mitochondrial complex I inhibitors (such as acetogenins and quinolines contained in Annonaceae, as well as rotenoids) are implicated in PSP-like brain injuries.[10]

Pathophysiology[edit]

The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles (NFTs), which are clumps of tau protein, a normal part of a brain cell's internal structural skeleton. These tangles are often different from those seen in Alzheimer's disease, but may be structurally similar when they occur in the cerebral cortex.[11] Their chemical composition is usually different, however, and is similar to that of tangles seen in corticobasal degeneration.[12] Tufts of tau protein in astrocytes, or tufted astrocytes, are also considered diagnostic. Unlike globose NFTs, they may be more widespread in the cortex.[13] Lewy bodies are seen in some cases, but whether this is a variant or an independent co-existing process is not clear, and in some cases, PSP can coexist with corticobasal degeneration, Parkinson's, and/or Alzheimer's disease, particularly with older patients.[14][15][16][17][18]

The principal areas of the brain affected are the:[citation needed]

  • basal ganglia, particularly the subthalamic nucleus, substantia nigra, and globus pallidus
  • brainstem, particularly the portion of the midbrain where "supranuclear" eye movement resides, as well as dopaminergic nuclei.
  • cerebral cortex, particularly that of the frontal lobes and the limbic system (similarly to frontotemporal degeneration)
  • dentate nucleus of the cerebellum
  • spinal cord, particularly the area where some control of the bladder and bowel resides

Some consider PSP, corticobasal degeneration, and frontotemporal dementia to be variations of the same disease.[19][20] Others consider them separate diseases.[21][22][23] PSP has been shown occasionally to co-exist with Pick's disease.[24]

Diagnosis[edit]

Person with progressive dementia, ataxia, and incontinence. A clinical diagnosis of normal-pressure hydrocephalus was entertained. Imaging did not support this, however, and on formal testing, abnormal nystagmus and eye movements were detected. A sagittal view of the CT/MRI scan shows atrophy of the midbrain, with preservation of the volume of the pons. This appearance has been called the "hummingbird sign" or "penguin sign". Also, atrophy of the tectum is seen, particularly the superior colliculi. These findings suggest the diagnosis of progressive supranuclear palsy.[25]

MRI is often done to diagnose PSP. MRI may show atrophy in the midbrain with preservation of the pons giving a "hummingbird" sign appearance and Mickey Mouse sign.[26]

Types[edit]

Based on the pathological findings in confirmed cases of PSP, it is divided into the following categories:

  • classical Richardson syndrome (PSP-RS)[citation needed]
  • PSP-parkinsonism (PSP-P) and PSP-pure akinesia with gait freezing (PSP-PAGF)[citation needed]
  • frontal PSP, PSP-corticobasal syndrome (PSP-CBS), PSP-behavioural variant of frontotemporal dementia (PSP-bvFTD) and PSP-progressive non-fluent aphasia (PSP-PNFA)[27]
  • PSP-C
  • PSP induced by Annonaceae[28]

Richardson syndrome is characterized by the typical features of PSP. In PSP-P features of Parkinson’s Disease overlap with the clinical presentation of PSP and follows a more benign course. In both PSP-P and PSP- PAGF distribution of abnormal tau is relatively restricted to the brain stem. Frontal PSP initially presents with behavioral and cognitive symptoms, with or without ophthalmoparesis and then evolve into typical PSP.[7] The phenotypes of PSP-P and PSP-PAGF are sometimes referred as the ‘brain stem’ variants of PSP, as opposed to the ‘cortical’ variants which present with predominant cortical features including PSP-CBS, PSP-bvFTD, and PSP-PNFA.[29] Cerebellar ataxia as the predominant early presenting feature is increasingly recognized as a very rare subtype of PSP (PSP-C) which is associated with severe neuronal loss with gliosis and higher densities of coiled bodies in the cerebellar dentate nucleus.[30]

Differential diagnosis[edit]

PSP is frequently misdiagnosed as Parkinson's disease because they both involve slowed movements and gait difficulty, with PSP being one of a collection of diseases referred to as Parkinson plus syndromes. Both Parkinson's and PSP have an onset in late middle age and involve slowing and rigidity of movement. However, several distinguishing features exist. Tremor is very common with Parkinson's, but rare with PSP. Speech and swallowing difficulties are more common and severe with PSP and the abnormal eye movements of PSP are essentially absent with PD.[31] A poor response to levodopa along with symmetrical onset can also help differentiate PSP from PD.[32] Patients with the Richardson variant of PSP tend to have an upright posture or arched back, as opposed to the stooped-forward posture of other Parkinsonian disorders, although PSP-Parkinsonism (see below) can demonstrate a stooped posture.[33] Early falls are also more common with PSP, especially with Richardson syndrome.[34]

PSP can also be misdiagnosed as Alzheimer's disease because of the behavioral changes.[35]

Chronic traumatic encephalopathy shows many similarities with PSP.[citation needed]

Current management[edit]

Supportive therapies[edit]

No cure for PSP is known, and management is primarily supportive. PSP cases are often split into two subgroups, PSP-Richardson, the classic type, and PSP-Parkinsonism, where a short-term response to levodopa can be obtained.[36] Dyskinesia is an occasional but rare complication of treatment.[37] Amantadine is also sometimes helpful.[38] After a few years the Parkinsonian variant tends to take on Richardson features.[39] Other variants have been described.[40][41][42][43] Botox can be used to treat neck dystonia and blepharospasm, but this can aggravate dysphagia.[44]

Two studies have suggested that rivastigmine may help with cognitive aspects, but the authors of both studies have suggested a larger sampling be used.[45][46] There is some evidence that the hypnotic zolpidem may improve motor function and eye movements, but only from small-scale studies.[47][48]

Rehabilitation[edit]

Patients with PSP usually seek or are referred to occupational therapy, speech-language pathology for motor speech changes typically a spastic-ataxic dysarthria, and physical therapy for balance and gait problems with reports of frequent falls.[49] Evidence-based approaches to rehabilitation in PSP are lacking and, currently, the majority of research on the subject consists of case reports involving only a small number of patients.[citation needed]

Case reports of rehabilitation programs for patients with PSP generally include limb-coordination activities, tilt-board balancing, gait training, strength training with progressive resistive exercises, and isokinetic exercises and stretching of the neck muscles.[49] While some case reports suggest that physiotherapy can offer improvements in balance and gait of patients with PSP, the results cannot be generalized across all PSP patients, as each case report only followed one or two patients.[49] The observations made from these case studies can be useful, however, in helping to guide future research concerning the effectiveness of balance and gait training programs in the management of PSP.[citation needed]

Individuals with PSP are often referred to occupational therapists to help manage their condition and to help enhance their independence. This may include being taught to use mobility aids.[50][51] Due to their tendency to fall backwards, the use of a walker, particularly one that can be weighted in the front, is recommended over a cane.[50] The use of an appropriate mobility aid helps to decrease the individual’s risk of falls and makes them safer to ambulate independently in the community.[51]Due to their balance problems and irregular movements, individuals need to spend time learning how to safely transfer in their homes and in the community.[50] This may include rising from and sitting in chairs safely.[51]

Due to the progressive nature of this disease, all individuals eventually lose their ability to walk and will need to progress to using a wheelchair.[50] Severe dysphagia often follows, and at this point death is often a matter of months.[36]

Experimental treatments[edit]

Drugs targeting the tau protein offer a promising avenue for therapeutic intervention. The growth factor davunetide was recently trialed in patients to prevent hyperphosphorylated, insoluble forms of tau, however it was unable to show efficacy possibly due insufficient CNS penetration.[52] Antisense therapy has shown efficacy in several other human neurodegenerative disorders and has recently been shown to substantially extend lifespan in animals with PSP.[53] Biogen and Ionis Pharmaceuticals currently are investigating a tau-lowering antisense therapy for Alzheimer's disease and frontotemporal dementia which could also have applicability to PSP.[54]

Prognosis[edit]

No effective treatment or cure has been found for PSP, although some of the symptoms can respond to nonspecific measures. The poor prognosis is predominantly attributed to the serious impact this condition has on the quality of life.[3] The average age at symptoms onset is 63 and survival from onset averages seven years with a wide variance.[55] Pneumonia is a frequent cause of death.[56]

History[edit]

In 1877, Charcot described a 40-year-old woman who had rigid-akinetic parkinsonism, neck dystonia, dysarthria, and eye-movement problems. Chavany and others reported the clinical and pathologic features of a 50-year-old man with a rigid and akinetic form of parkinsonism with postural instability, neck dystonia, dysarthria, and staring gaze in 1951. Progressive supranuclear palsy was first described as a distinct disorder by neurologists John Steele, John Richardson, and Jerzy Olszewski in 1963.[1][57][58][59] They recognized the same clinical syndrome in 8 patients and described the autopsy findings in 6 of them in 1963.[citation needed]

Progressive supranuclear palsy was not a “new” disease in 1963, as 22 well-documented case reports had been identified in the neurologic literature between 1877 and 1963.[60] The unique frontal lobe cognitive changes of progressive supranuclear palsy (apathy, loss of spontaneity, slowing of thought processes, and loss of executive functions) were first described by Albert and colleagues in 1974.[61]

Societies[edit]

There are several organizations around the world that support PSP patients and the research into PSP and related diseases, such as corticobasal degeneration (CBD) and multiple system atrophy (MSA).

  • Canada: PSP Society of Canada, a federally registered non-profit organization which serves patients and families dealing with PSP, CBD and MSA, set up in 2017 through the help of CurePSP in the USA[62]
  • France: Association PSP France, a nonprofit patient association set up in 1996 through the help of PSPA in the UK. It also gives support to French speaking patients in Quebec, Morocco, Algeria, Belgium and Lebanon[63]
  • UK: PSPA, a national charity for information, patient support and research of PSP and CBD, set up in 1995[64]
  • Ireland: PSPAI, a body which aims to get PSP better known[65]
  • US: CurePSP, a nonprofit organization for promoting awareness, care and research of PSP, CBD, MSA "and other prime of life neurodegenerative diseases"[66]

In popular culture[edit]

In the 2020 American musical comedy-drama television series, Zoey's Extraordinary Playlist, the title character's father (Mitch Clarke, played by Peter Gallagher) has PSP and dies in the final episode of the first season.[67]

American singer Linda Ronstadt was diagnosed with PSP in 2019, subsequent to an initial diagnosis of Parkinson's disease in 2014.[68]

See also[edit]

  • Lytico-bodig disease (Parkinsonism-Dementia Complex of Guam)
  • Annonacin

References[edit]

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Classification
D
  • ICD-10: G23.1
  • ICD-9-CM: 333.0
  • OMIM: 601104
  • MeSH: D013494
  • DiseasesDB: 10723
External resources
  • MedlinePlus: 000767
  • eMedicine: neuro/328
  • Patient UK: Progressive supranuclear palsy
  • Orphanet: 683