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Neurology

Symptoms and Physical Changes in HPHA

At a Glance

HPHA combines Parkinson-like movement symptoms—usually slowness and stiffness on one side—with smaller size or structural asymmetry on that same side. Tremor, weakness, clumsiness, dystonia, and balance problems may also occur, and symptoms can remain one-sided for years.

Hemiparkinsonism-Hemiatrophy Syndrome (HPHA) is characterized by a unique combination of physical changes that affect one side of the body. While it shares some symptoms with typical Parkinson’s disease, its presentation involves more than just movement slowness. The hallmark of the condition is the presence of parkinsonism (movement symptoms) on the same side of the body that shows hemiatrophy (smaller size, bone asymmetry, or muscle wasting) [1][2].

Because this syndrome is heterogeneous, your symptoms may look different from another person’s. For instance, some individuals have clear size differences that are visible to the naked eye, while others may only show “atrophy” on a brain scan without obvious changes in their physical appearance [3][4].

The “Hemi-Parkinson” Symptoms

The movement-related symptoms of HPHA are almost always confined to one side of the body at the start, and they often stay on that side for a much longer time than in typical Parkinson’s disease, though progression can occur [5][4]. These symptoms typically include:

  • Bradykinesia (Slowness of Movement): You may notice a lack of spontaneous movement, such as a reduced arm swing when walking or a mask-like facial expression [2]. Simple tasks like buttoning a shirt or using a computer mouse can become frustratingly slow.
  • Rigidity: This is a specific type of muscle stiffness where your limbs feel heavy or “lead-like” when moved by someone else [2]. This stiffness can cause discomfort or a deep aching sensation in the affected shoulder or hip.
  • Tremor: While not present in everyone, some patients experience a resting tremor—a rhythmic shaking that occurs when the hand or leg is relaxed but often stops when you start a task [4].

Understanding Hemiatrophy (Physical Asymmetry)

The term hemiatrophy refers to the smaller size of one side of the body. In HPHA, this wasting usually occurs on the same side as the movement symptoms (ipsilateral) [1][4]. This can manifest in several ways, and importantly, can reflect reduced growth of bone or soft tissue, congenital differences, or disuse, not just muscle wasting:

  • Limb Differences: One arm or leg may be structurally smaller or shorter than the other [4]. You might notice that one shoe feels looser than the other, or that your watch strap needs to be tighter on one wrist.
  • Distal Wasting: In some cases, the size difference is most noticeable in the “distal” parts of the body—the hands or the feet [4].
  • Facial or Truncal Involvement: Although less common, the asymmetry can sometimes affect the muscles of the face or the trunk, leading to a subtle tilt or a slight difference in facial features [5].

It is important to note that the brain often shows the opposite pattern: if your right side is smaller and slower, a brain scan may show that the left side of your brain is smaller (cerebral hemiatrophy) [3][2].

Beyond the Basics: Weakness and Dystonia

Unlike typical Parkinson’s disease, where true muscle weakness is less common early on, people with HPHA often report a variety of other motor challenges:

  • Weakness and Clumsiness: Patients often describe a sense of reduced strength or a lack of coordination in the affected hand or leg [2][3]. This might feel like your leg is “dragging” or your hand is “fumbling” with small objects.
  • Dystonia: This involves involuntary muscle contractions that cause repetitive movements or abnormal postures [3]. A common example in HPHA is a dystonic hand posture, where the fingers may curl or the wrist may twist into an awkward position when you try to use it [3].
  • Reflex Changes: Doctors may find reflex changes during an exam [2]. These findings must be interpreted in the context of your overall examination and evaluated for other causes.

How Symptoms Progress

The clinical presentation of HPHA is often observed for its stability in case reports. While typical Parkinson’s tends to spread from one side to the other over time, HPHA has been reported to remain predominantly unilateral for years or even decades in some patients [5][2]. However, this is not a universal rule; some patients do eventually experience symptoms on the other side [4]. Slowness, balance issues, and a loss of manual dexterity are common long-term challenges reported by patients [6].

Common questions in this guide

What are the main symptoms of hemiparkinsonism-hemiatrophy syndrome?
HPHA typically causes movement symptoms such as slowness, stiffness, and sometimes a resting tremor on one side of the body. The same side may be smaller or show bone, soft-tissue, or muscle differences, with weakness, clumsiness, dystonia, balance problems, or reduced hand dexterity also possible.
Does HPHA always affect only one side?
HPHA usually begins with parkinsonism and physical asymmetry on the same side and may remain predominantly one-sided for years or decades. Some people later develop symptoms on the other side, so one-sided symptoms are common but not an absolute rule.
What does hemiatrophy mean in HPHA?
Hemiatrophy means that one side of the body is smaller or structurally different. It can involve a limb, hand, foot, face, or trunk and may reflect differences in bone or soft tissue, growth, or use rather than muscle loss alone.
How can I tell weakness from parkinsonian slowness?
Weakness means reduced force, while bradykinesia means that movement is slow or difficult to initiate even when strength may be present. A clinician uses your history and neurologic examination to distinguish them because the sensations can overlap.
Can HPHA cause dystonia or unusual hand postures?
Yes. HPHA can cause dystonia, in which involuntary muscle contractions make the fingers curl or the wrist twist into an unusual position, especially during use. Tell your clinician if these postures are painful, interfere with tasks, or are becoming more frequent.
Why might a brain scan show changes on the opposite side?
The brain side controlling movement is often opposite to the affected side of the body. Therefore, if the right side of the body is smaller and slower, imaging may show reduced size on the left side of the brain; scan findings must be interpreted with the examination.

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References

References (6)
  1. 1

    Hemiparkinsonism-hemiatrophy syndrome.

    Ayromlou H, Najmi S, Arami MA

    Archives of Iranian medicine 2011; (14(2)):152-4 doi:011142/AIM.0017.

    PMID: 21361726
  2. 2

    [Hemiparkinsonism-Hemiatrophy Syndrome with Hypoperfusion in Basal Ganglia: A Case Report].

    Ma B, Wang LH, Zhang YN

    Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science edition 2021; (52(3)):531-533 doi:10.12182/20210560106.

    PMID: 34018377
  3. 3

    Subcortical and brainstem hemiatrophy accompanied by iron deposition in a patient with hemiparkinsonism-hemiatrophy syndrome: a case report.

    Chung SJ

    BMC neurology 2021; (21(1)):51 doi:10.1186/s12883-021-02080-4.

    PMID: 33535984
  4. 4

    Neuropathologic findings in a patient with hemiparkinsonism and hemiatrophy syndrome.

    Nakamura M, Tsuge A, Miyake K, et al.

    Neuropathology : official journal of the Japanese Society of Neuropathology 2024; (44(6)):432-439 doi:10.1111/neup.12994.

    PMID: 38972833
  5. 5

    Hemiparkinsonism or Hemidystonia With Hemiatrophy Syndrome: A Case Series With Follow-Up.

    He C, Zhang P, Li Y, et al.

    Frontiers in neuroscience 2020; (14()):64 doi:10.3389/fnins.2020.00064.

    PMID: 32116511
  6. 6

    Bidirectionality of the dentato-rubro-thalamo-cortical tract allows concurrent hypoperfusion in ipsilateral cerebellum and contralateral cerebral hemisphere: A case report.

    He HC, Hsu MC, Hsu CS, et al.

    Medicine 2018; (97(40)):e12590 doi:10.1097/MD.0000000000012590.

    PMID: 30290625

This page describes HPHA symptoms and physical asymmetry for informational purposes only and does not constitute medical advice. A neurologist should assess one-sided movement changes, weakness, or atrophy in your specific situation.