This 46-year-old man presented with a 20-year history of progressive distal wasting and weakness of the right hand and forearm muscles. This 46-year-old.

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 Hirayama Disease.  Aka Juvenile Muscular Atrophy of the Distal Upper Extremity  Rare disease that affects predominantly males in their 2 nd or early.

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Muscle cramps and a raised creatine kinase. Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology.

Teaching NeuroImages A 54-year-old man with progressive muscle weakness, hand tremor, tongue and perioral fasciculation Neurology Resident and Fellow Section.

A 17 year old male with tremors and wasting of both hands

Fig. 3.Lumbosacral spine magnetic resonance imaging (MRI) of 48-year-old woman who presented with low back pain. A. Sagittal T2-weighted image shows asymmetric.

Hirayama Disease Neuroimaging Clinics

Figure 3 3D magnetic resonance neurography

Figure 2 Evolution of MRI abnormalities in faciobrachial dystonic seizures Axial fluid- attenuated inversion recovery (FLAIR)-weighted images from patient.

Figure Facial photograph during headache attack and brain and upper cervical cord MRI Facial photograph during headache attack and brain and upper cervical.

A 20-year-old man with HD. A, Neutral axial gradient-echo image at the C5 level demonstrates subtle bilateral LOA along the lateral aspects of the lamina.

A, Brachial neuritis with long thoracic nerve neuritis.

REFLEX and REFLEXION study design.

OCT left eye (case 3). OCT showed bilateral foveal atrophy (white arrows), with an island of preserved retina in the left fovea (yellow arrow; only left.

Figure 1 MRI head in faciobrachial dystonic seizures (A) Axial fluid-attenuated inversion recovery image from patient 3 in table 2 shows T2-weighted hyperintensity.

A 42-old-woman with tingling and numbness in the ulnar side of the right hand. A 42-old-woman with tingling and numbness in the ulnar side of the right.

(A) This 54 year old man with the flail arm syndrome has severe wasting of the arms causing profound weakness. (A) This 54 year old man with the flail.

TOS with C8 neuropathy. TOS with C8 neuropathy. A 17-year-old girl (trumpet player) with left arm and hand weakness in an ulnar distribution, exacerbated.

Multifocal motor neuropathy with conduction block.

Brain and spine MR imaging of a 48-year-old woman with Zika virus infection and encephalitis and myelitis. Brain and spine MR imaging of a 48-year-old.

The MR scan of brain of our case vignette patient showing significant occipital lobe atrophy (especially left sided) with parietal lobe involvement as.

(A) EEG of a 75 year old woman with adult onset myoclonic epilepsy (see text, case 1), showing generalised spike and wave and polyspike and wave discharges.

Pseudoptosis. Pseudoptosis. This man presented with photophobia and difficulty elevating the right side of his forehead. The photograph shows his normal.

Multifocal motor neuropathy with conduction block.

Neurotmesis with suprascapular nerve severance and axillary neuropathy

Spine MR imaging of a 35-year-old man with Zika virus infection and Guillain-Barré syndrome presenting with progressive ascending paralysis that evolved.

The same patient as in Fig 3.

Neurology Resident and Fellow Section

Neuroimaging and macroscopic features.

Figure Spinal cord imaging (A, B) Sagittal and axial T2-weighted cervical spine MRI demonstrating hyperintensities in the central gray matter of patient.

The same patient as in Fig 3.

T2 weighted (T2W), FLAIR (fluid attenuated inversion recovery), and T1W images demonstrate severe head atrophy with ex vacuo dilatation of the frontal.

Flexion cervical spine MRI in Hirayama disease showing expansion of the dural venous plexus with presumed chronic ischaemic damage preferentially involving.

Persistent weakness of right lower limb 2 months after EV71 infection in a 16-month-old infant. Persistent weakness of right lower limb 2 months after.

(A) Axial CT scan of head at presentation, showing a right occipital hypodense lesion. (A) Axial CT scan of head at presentation, showing a right occipital.

T2 weighted sagittal MRI scans of the cervical spine.

Sagittal T2-weighted MR scan of spine of case 1 (A) and case 2 (C), showing intramedullary signal hyperintensity at T11/12 in case 2 (C). Sagittal T2-weighted.

Axial cervical spine anatomy.

Figure A 57-year-old man with relapsing-remitting MS (RRMS) and new-onset ataxia A 57-year-old man with relapsing-remitting MS (RRMS) and new-onset ataxia.

The superior quality of MRI over CT is demonstrated in this figure.

(A) MR scan of brain from an 82-year-old woman who presented with recurrent episodes of sudden onset needles affecting the face, gum and hand, with facial.

Sample MR images obtained acutely from patients 1 to 6: axial T2 weighted (DWI in case 4) on the left, coronal FLAIR on the right of each panel. Sample.

A–C, Sagittal T1-weighted (A), sagittal T2-weighted (B), and axial T2-weighted (C) MR images of the cervical spine in a patient with severe myelopathy.

A 50-year-old woman with nonenhancing WHO grade II diffuse astrocytoma

Clinical overall score (COS), (A); cold detection thresholds (CDT), (B); warm detection thresholds (WDT), (C); and vibration thresholds (VT), (D) in patients.

Heavily T2-weighted MRI obtained with high resolution, showing multiple enlarged VRS, visible as well-demarcated CSF like structures, which, dependent.

Axial MRI of a 46 year old man with secondary progressive MS showing a large left sided periventricular lesion which is hyperintense with (A) T2 weighted.

(A) Axial FLAIR MRI reveals multiple areas of high signal intensity (arrows) in leptomeninges. (A) Axial FLAIR MRI reveals multiple areas of high signal.

Progression of autonomic symptoms including hypohidrosis (A), faintness (B), syncope (C), constipation (D), urinary dysfunction (E), and respiratory disturbance.

A patient with Cori-Forbes disease who had childhood hepatomegaly and hypoglycaemic episodes; despite a liver biopsy, there was no diagnosis. A patient.

Left side: normal short latency somatosensory evoked potentials (SSEPSs) after stimulation of the median nerve (top picture) and posterior tibial nerve.

Sagittal midline T1-weighted (A) and coronal fluid-attenuated (B) inversion recovery (C) images demonstrate evidence of progressive cerebellar atrophy.

Examples of the visual rating scale for the medial and lateral temporal lobe on MR coronal images displayed conventionally with the letter on the right.

(A) High intensity lesions in the left dorsolateral midbrain on T2 weighted magnetic resonance imaging in case 1. (A) High intensity lesions in the left.

[123I]-FP-CIT (DaTSCAN) images demonstrating: (top left) normal tracer uptake in the putamen and caudate nuclei; then progressively decreasing uptake in.

Kaplan-Meier table analysis of patients with corticobasal degeneration after onset of symptoms; the y axis refers to proportion of patients who are alive.

(A) Patient with acute PFP on the 4th day showed an abnormal dyphagia limit of 5 ml from paretic left sided swallowing (arrows denote second swallows).

Bilateral opercular polymicrogyria.

Axial T1-weighted MRI of the thigh (A) and lower leg (B) for case 1 shows fatty infiltration of the quadriceps and adductor muscles (L2, 3 and 4 myotomes;

Short-interval follow-up cervical MR imaging of a 67-year-old male ASIA A patient with SCI. T2-weighted FSE images were obtained from an initial MR imaging.

MR scan of brain fluid-attenuated inversion recovery (FLAIR) (A) and short tau inversion recovery (STIR) (B, C) showing asymmetrical hyperintensities affecting.

MR scans of brain and spine: (A) sagittal T2 image showing signal change in the posterior spinal cord between C3 and T6. MR scans of brain and spine: (A)

Congenital cortical malformation.

Axial magnetic resonance imaging (MRI) of a 30 year old man with relapsing remitting multiple sclerosis (MS) showing multiple periventricular lesions:

Axial 1mm thick gadolinium enhanced T1 weighted image through the geniculate ganglion (white arrow). Axial 1mm thick gadolinium enhanced T1 weighted.

A 19-year-old man with a nonenhancing WHO grade III anaplastic astrocytoma. A 19-year-old man with a nonenhancing WHO grade III anaplastic astrocytoma.

Radiation plexopathy. Radiation plexopathy. A 67-year-old woman with a history of left breast carcinoma and radiation treatment 20 years ago who presented.

Axial T2-weighted MRI. (A and B) Dot-like hyperintensities characteristic of enlarged perivascular spaces (EPVS) in the basal ganglia in a patient with.

Patient 4, a 72-year-old man presenting with headache, dysphagia, and progressive hoarseness. Patient 4, a 72-year-old man presenting with headache, dysphagia,

Figure 1 MRIs MRIs (A and B) Axial FLAIR images of the brain demonstrate multifocal parenchymal lesions including the right hippocampus, right midbrain,

Perimysial pathology in patients with selectively elevated aldolase.

Presentation transcript:

This 46-year-old man presented with a 20-year history of progressive distal wasting and weakness of the right hand and forearm muscles. This 46-year-old man presented with a 20-year history of progressive distal wasting and weakness of the right hand and forearm muscles. Symptoms developed in the left hand 5 years prior to presentation. Upper limb reflexes were depressed. Needle electromyography revealed chronic neurogenic changes in clinically affected muscles. There were no sensory abnormalities. (A and B) Asymmetrical wasting of the hands and forearm affecting C7-T1 musculature with striking preservation of brachioradialis in the right upper limb; (C) sagittal T2-weighted STIR image demonstrating a linear hyperintensity within the cervical cord at C6 and C7 associated with cord atrophy; (D) axial T2-weighted image with ‘snake eyes’ appearance in the anterior horns. STIR, Short TI Inversion Recovery. Nidhi Garg et al. J Neurol Neurosurg Psychiatry 2017;88:474-483 ©2017 by BMJ Publishing Group Ltd