Hemiparkinsonismhemiatrophy

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Hemiparkinsonism-hemiatrophy (HPHA) syndrome was first described by Klawans in 1981 (87), who described four individuals with known hemiatrophy with narrow extremities on one side who developed delayed-onset hemiparkinsonism between ages 31 and 40 with tremor on the same side as the hemiatrophy along with rigidity, akinesia, and dystonia, but no evidence of hypomimia, or abnormal posture or lack of postural reflexes. Their symptoms remained unilateral between 5 and 35 yr after onset of illness. They did not respond well to levodopa. HPHA can be differentiated from idio-pathic PD by the clinical features of hemiatrophy, asymmetric parkinsonism more prominent on the side of hemiatrophy, dystonia, early age at onset, history of birth injury, and slow progression of the disease. Mean age of onset of parkinsonism in HPHA is 43.7 yr (range 31-61), and mean duration of symptoms was 9.4 yr (range: 1-35 yr). The first symptom in majority of cases is tremor, followed by bradykinesia and dystonia. In some cases the tremor may become bilateral (88-90). Greene et al. (90) have reported dopa-responsive dystonia in some patients with HPHA. In one case report of a 47-yr-old woman with HPHA, the patient developed unusual symptoms of exertional- induced weakness of the right ankle followed by prolonged inversion and dorsiflexion of her foot (91). The symptoms later evolved into tremor, stiffness, and lack of dexterity of muscles on the right side. She had good

Table 2

Showing the Clinical Features of Nondegenerative Atypical Parkinsonian Disorders that Help in Diagnosis

Parkinsonian Disorder Distinguishing Clinical Features Supportive MRI Findings

Vascular parkinsonism

MPTP-induced parkinsonism Manganese-induced parkinsonism

Carbon monoxide-induced parkin-

Cyanide-induced parkinsonism

Methanol-induced parkinsonism Ethanol-induced parkinsonism

HIV-related parkinsonism

SSPE-related parkinsonism Mycoplasma-related parkinsonism

Bilateral striatopallidodentate calcinosis (Fahr's disease)

Acute, delayed, or insidious onset with bradykinesia, rigidity, short stepped gait, with or without dementia, postural instability lower body involvement. Patients have freezing of gait, absence of tremor, poor levodopa responsiveness. Some patients have pseudobulbar affect. High index of suspicion in presence of vascular risk factors, and an older patient with atypical clinical presentation. Features similar to idiopathic PD in presence of toxin exposure. Parkinsonism with postural tremor, dystonia, cock-walk, postural instability in presence of exposure to manganese

Masked facies, short stepped gait, rigidity hypolinesia, cognitive impairment, mutism postural tremor, urinary or fecal incontinence, dystonia, emotional lability, postural instability Parkinsonism, with dystonia, apraxia of eyelid opening, with temporal relation to the onset of symptoms and exposure to cyanide Metabolic acidosis, coma, parkin-sonism, especially rigidity, and bradykinesia

Transient parkinsonism seen during alcohol withdrawal, developing a few days after consumption of the last drink. Most patients have previous transient episodes. Rapid progression of parkinsonian symptoms tremor, in presence of HIV with or without related CNS infections such as toxoplasmosis Parkinsonism in presence of other abnormalities suggestive of SSPE Parkinsonism with flulike symptoms, and dystonia, with elevated mycoplasma antibody levels Parkinsonism, cognitive impairment, celebellar signs, speech abnormalities, pyramidal signs, psychiatric features and sensory abnormalities

Subcortical ischemic changes on brain MRI

No specific changes

T1 hyperintensity in the striatum and globus pallidus on brain MRI

T2 hyperintensities in the white matter on brain MRI

T2 hyperintensities in the putamen hemorrhagic necrosis in the cerebral cortex, especially in the sen-sorimotor cortex

Subcortical white matter changes

No specific changes

MRI abnormalities in case of CNS opportunistic infections such as toxoplasmosis, CMV (cytomegalovirus)

Lesions in caudate and putamen, but sparing the blobus pallidus and thalamus

Increased T1 and T2 signal in the basal ganglia

MRI findings of calcium in the subcortical white matter and basal ganglia

Table 2 (Continued)

Parkinsonian Disorder

Distinguishing Clinical Features

Supportive MRI Findings

Post-anoxic parkinsonism

Drug-induced parkinsonism

Peripherally induced parkinsonism Developmental parkinsonism

Hemiatrophy-hemiparkinsonism

Structural lesions causing parkinsonism

Parkinsonism owing to multiple sclerosis

Parkinsonism with or without dystonia weeks to months after the ischemic event

Parkinsonism in association with other involuntary movements including bucco-lingulo-mastica-tory syndrome, dystonia, stereotypes, akathisia and gait disturbance with history of exposure todopamine receptor-blocking drug

Tremor-dominant parkinsonism following injury Parkinsonism following some insult in utero, or immediate postnatal period in association with tremor, cognitive dysfuncion, behavioral abnormalities, headaches, and strabismus Hemiatrophy in association with hemiparkinsonism dystonia, but without postural instability Tends to remain unilateral for 535 yr after onset of parkinsonian symptoms; Early age at onset, history of birth injury, slow progression of the disease are very typical features. Parkinsonism in presence of tumors, hemorrhage typically contralateral to the lesion and sometimes ipsilateral to the lesion Parkinsonian features in presence of other symptoms of multiple sclerosis

T1 hyperintensities in the basal ganglia bilaterally

No specific changes

No specific changes described

Abnormal in presence of clear ischemic event

Hemiatrophy on MRI of the brain, may have associated skull asymmetry

MRI changes are usually consistent with the space occupying lesion.

MRI lesions in substantia nigra, and in the cervicomedullary junction response of the dystonia parkinsonism to levodopa. Her MRI showed atrophy in the substantia nigra. The unusual constellation of exertional dystonia with parkinsonism and hemiatrophy demonstrates the wide range of clinical features seen in this syndrome. An unusual case of brain hemihypoplasia with contralateral hemiatrophy and hemiparkinsonism was described (92). MRI of the brain showed skull and encephalic asymmetry and hypoplasia of the right side and the 99mTc ECD SPECT showed global hypoperfusion of the right hemisphere.

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